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‘¢ŒŒŠķŽīį‡@`Ž”—ĆƒKƒCƒhƒ‰ƒCƒ“

ƒKƒCƒhƒ‰ƒCƒ“•¶’†‚Ģ•¶Œ£”Ō†‚©‚ēCŠY“–‚·‚镶Œ£ƒŠƒXƒg‚ÖƒŠƒ“ƒN‚³‚ź‚Ü‚·

–ŚŽŸF


ID”’ŒŒ•a

1
  ‹}«œ‘«”’ŒŒ•a
iacute myeloid leukemiaFAMLj

Ÿ‘˜_

1DAML ‚Ģ•a‘Ō‚ĘŽ”—Ć

‹}«œ‘«”’ŒŒ•aiacute myeloid leukemiaFAMLj‚Ķ•Ŗ‰»E¬n”\‚ŖįŠQ‚³‚ź‚½—cŽįœ‘Œn×–E‚ĢƒNƒ[ƒiƒ‹‚ČŽ©—„«‘B‚š“Į’„‚Ę‚·‚鑽—l«‚É•x‚ŽŒŒ‰tŽīį‡‚Å‚ ‚éBœ‘‚É‚Ø‚Æ‚é”’ŒŒ•a×–E‚ĢˆŁķ‚Č‘B‚ĢŒ‹‰ŹC³ķ‚Č‘¢ŒŒ‹@”\‚Ķ’˜‚µ‚­‘jŠQ‚³‚źC”’ŒŒ‹…Œø­C•nŒŒCŒŒ¬”ĀŒø­‚É”ŗ‚¤‚³‚Ü‚“‚Ü‚ČĒó‚š’ę‚·‚éB“KŲ‚ČŽ”—Ć‚Ŗ‚Č‚³‚ź‚Č‚¢ź‡‚ĶCŠ“õĒ‚āoŒŒ‚É‚ę‚č’ZŠśŠŌ‚Å’vŽ€“I‚Ę‚Č‚éd“Ä‚ČŽ¾Š³‚Å‚ ‚éB

‰”­AML ‚É‘Ī‚·‚éŠī–{“I‚ČŽ”—Ɛķ—Ŗ‚ĶŽ”–ü‚š–ŚŽw‚µ‚½‹­—Ķ‚ȉ»Šw—Ć–@‚Å‚ ‚čC‘½Ü•¹—p—Ć–@‚ŖŠī–{‚Ę‚Č‚éB‚µ‚©‚µC‚»‚Ģ“K‰ž‚Ķ‰»Šw—Ć–@‚É‚ę‚é‘ŸŠķ“Ő«‚ā‡•¹Ē‚É‘Ļ‚¦‚ē‚ź‚é‚©‚š”N—īC‘ŸŠķ‹@”\C‘Sgó‘Ō‚Č‚Ē‚É‚ę‚Į‚ĐTd‚©‚ĀŒµ–§‚É”»’f‚·‚é•K—v‚Ŗ‚ ‚éi•\1j1j2jBAML ‚É‘Ī‚·‚鉻Šw—Ć–@‚ĶŠ°‰š“±“ü—Ć–@‚ʁCŠ°‰š‚Ŗ“¾‚ē‚ź‚½Œć‚ɍs‚¤Š°‰šŒć—Ć–@‚©‚ē‚Č‚éB‰»Šw—Ć–@‚Ģ‚Ż‚Å‚Ķ—ĒD‚Č’·Šś—\Œć‚Ŗ“¾‚ē‚ź‚Č‚¢Ē—į‚É‘Ī‚µ‚Ä‚ĶC‘ęˆźŠ°‰šŠś‚Å“ÆŽķ‘¢ŒŒŠ²×–EˆŚA‚Ŗ“K‰ž‚Ę‚Č‚éB

Š°‰š“±“ü—Ć–@‚É‘Ī‚·‚é•s‰ž—į‚āCŠ®‘SŠ°‰šicomplete remissionFCRj‚É“ž’B‚µ‚½‚ą‚Ģ‚ĢC‚»‚ĢŒćÄ”­‚š‚«‚½‚µ‚½Ē—į‚ĶCÄ”­E“ļŽ”—į‚Ę‚µ‚Ä‹~‰‡—Ć–@‚Ŗ•K—v‚Ę‚Č‚éB‚µ‚©‚µCÄ”­E“ļŽ”—į‚É‚Ø‚¢‚Ä‚Ķ‰»Šw—Ć–@‚Ģ‚Ż‚Å‚ĢŽ”–ü‚ĶŠś‘Ņ‚µ‚Ŗ‚½‚¢‚½‚߁C‰Ā”\‚ȏĒ—į‚Å‚Ķ“ÆŽķ‘¢ŒŒŠ²×–EˆŚA‚Ŗ“K‰ž‚Ę‚Č‚éB

‚—īŽŅAML ‚Å‚ĶC‘ŸŠķ‹@”\‚Č‚Ē‚ĢŠ³ŽŅ‘¤—vˆö‚É‚ę‚čCŽį”N¬l‚Ę“Æ“™‚ĢŽ”—Ć‹­“x‚šŽ‚Ā‰»Šw—Ć–@‚šˆź—„‚ÉŽĄŽ{‚·‚邱‚Ę‚Ķ¢“ļ‚Å‚ ‚éB‘Sgó‘Ō‚ā‘ŸŠķ‹@”\‚Ŗ[•Ŗ‚É•Ū‚½‚ź‚Ä‚¢‚鏼‡‚É‚Ķ‰»Šw—Ć–@‚Ģ“K‰ž‚Ę‚Č‚é‚ŖCˆź”Ź“I‚ɍ‚—īŽŅAML ‚É‘Ī‚·‚鉻Šw—Ć–@‚ĶŽ”—ĆŠÖ˜A‡•¹Ē‚Ģ•p“xE’ö“x‚Ŗ‚‚­C‹­—Ķ‰»Šw—Ć–@‚Ģ“K‰ž‚ĶTd‚É”»’f‚µ‚Č‚Æ‚ź‚Ī‚Č‚ē‚Č‚¢B

•\1@‹­—Ķ‰»Šw—Ć–@“K‰ž‹K€1j2j
€–Ś Šī€
”N—ī 65 Ī–¢–ž
S‹@”\ ¶Žŗ‹ģo—¦iLVEFj50“ˆČć
”x‹@”\ PaO2M/ 60Torr ˆČć‚Ü‚½‚ĶSpO2 90“ˆČćiroom airj
ŠĢ‹@”\ ŒŒ“ƒrƒŠƒ‹ƒrƒ“2.0 mg/dL ˆČ‰ŗ
t‹@”\ ŒŒ“ƒNƒŒƒAƒ`ƒjƒ“Ž{ŻŠī€’l‚ĢćŒĄ‚Ģ1.5 ”{ˆČ‰ŗ
Š“õĒ §Œä•s”\‚ĢŠ“õĒ‚Ģ‡•¹‚Č‚µ

JALSGi“ś–{¬l”’ŒŒ•aŽ”—Ć‹¤“ÆŒ¤‹†ƒOƒ‹[ƒvj‚É‚Ø‚Æ‚é—Տ°‘ęⅢ‘ŠŽŽŒ±‚Å’č‚ß‚é“KŠi‹K€‚Č‚Ē‚šŽQl‚É‹­—Ķ‰»Šw—Ć–@‚šs‚¤‚É‚ ‚½‚čć‹L‹K€‚Ŗ–ŚˆĄ‚Ę‚Č‚é‚ŖCŠ³ŽŅ‚Ģ‘Sgó‘Ō‚ā‚»‚Ģ‘¼‚Ģ‡•¹Ē‚šl—¶‚µ‚Ä‘‡“I‚É”»’f‚·‚é•K—v‚Ŗ‚ ‚éB

2DAML ‚Ģf’f‚Ę•aŒ^•Ŗ—Ž

AML ‚Ģf’f‚ĶC①œ‘‚É‚Ø‚Æ‚é”’ŒŒ•a×–E‚Ģ‘¶ŻiWHO •Ŗ—Ž‚Å‚Ķ20“ˆČćCFAB •Ŗ—Ž‚Å‚Ķ30“ˆČćjC②”’ŒŒ•a×–E‚Ŗœ‘Œn‹NŒ¹‚Å‚ ‚邱‚ʁC③”’ŒŒ•a×–E‚ĢõF‘ĢŠjŒ^Eˆā“`Žq•ĻˆŁ‰šĶ‚É‚ę‚Į‚čs‚ķ‚źC‚»‚ĢŒćWHO •Ŗ—Ži2008j‚É‚ę‚Į‚Ä•aŒ^•Ŗ—Ž‚³‚ź‚éi•\2j3jB]—ˆ”Ä—p‚³‚ź‚Ä‚«‚½FAB •Ŗ—Ž‚Ķde novo AML ‚Ģ‚Ż‚š‘ĪŪ‚Ę‚µ‚Ä‚«‚½‚ŖCWHO •Ŗ—Ž‚Å‚ĶŽ”—ĆŠÖ˜AAML ‚ʍœ‘ˆŁŒ`¬‚ÉŠÖ˜A‚µ‚½•Ļ‰»‚š—L‚·‚éAML ‚Ø‚ę‚ŃAML ŠÖ˜A‘O‹ģ×–E«Žīį‡iœ‘“÷ŽīC‰č‹…Œ`Žæ×–E—lŽ÷ó×–EŽīį‡j‚šŠÜ‚Ž‚Ę‚Ę‚ą‚ɁC”½•œˆā“`ŽqˆŁķ‚š—L‚·‚éAML ‚Ęƒ_ƒEƒ“Ē‚É”ŗ‚¤œ‘‘BĒ‚šˆź‚Ā‚ĢƒJƒeƒSƒŠ[‚Ę‚µ‚Ä’č‹`‚µ‚Ä‚¢‚éBWHO •Ŗ—Ž‚Å‚Ķ‚±‚ź‚ēƒJƒeƒSƒŠ[‚ÉŠY“–‚µ‚Č‚¢Ē—į‚š•Ŗ—Ž•s”\‚ĢAMLiAML, not otherwise specifiedj‚Ę‚µ‚Ä‚¢‚é‚ŖC‚»‚Ģ×•Ŗ—Ž‚É‚ĶFAB •Ŗ—Ž‚É‚Ø‚Æ‚éŒ`‘ŌŠw“IE–ʉu‘gDŠw“If’f‚Ŗ—p‚¢‚ē‚ź‚é4jB

•\2@AML ‚ĢWHO •Ŗ—Ži2008j3j
Acute myeloid leukemia with recurrent genetic abnormalities
  • AML with t (8;21)(q22;q22); RUNX1-RUNX1T1
  • AML with inv (16)(p13.1q22) or t (16;16)(p13.1;q22);CBFB-MYH11
  • APL with t (15;17)(q22;q12); PML-RARA
     Variant RARA translocations
  • AML with t (9;11)(p22;q23); MLLT3-MLL
     Variant MLL translocations
  • AML with t (6;9)(p23;q34); DEK-NUP214
  • AML with inv (3)(q21q26.2) or t (3;3)(q21;q26.2); RPN1-EVI1
  • AMLimegakaryoblasticjwith t (1;22)(p13;q13); RBM15-MKL1
  • Provisional entity: AML with mutated NPM1
  • Provisional entity: AML with mutated CEBPA
Acute myeloid leukemia with myelodysplasia-related changes
Therapy-related myeloid neoplasms
Acute myeloid leukemia, not otherwise specified
  • AML with minimal differentiationiFAB: M0j
  • AML without maturationiFAB: M1j
  • AML with maturationiFAB: M2j
  • Acute myelomonocytic leukemiaiFAB: M4j
  • Acute monoblastic/monocytic leukemiaiFAB: M5j
  • Acute erythroid leukemiaiFAB: M6j
     Pure erythroid leukemia
     Erythroleukemia, erythroid/myeloid
  • Acute megakaryoblastic leukemiaiFAB: M7j
  • Acute basophilic leukemia
  • Acute panmyelosis with myelofibrosis
Myeloid sarcoma
Myeloid proliferations related to Down syndrome
Blastic plasmacytoid dendritic cell neoplasms

3DAML ‚Ģ—\ŒćˆöŽq

•W€“I‚ȉ»Šw—Ć–@‚šŽó‚Æ‚½Žį”N¬lAML ‘S‘Ģ‚Å‚ĶC70`80“‚ĢCR ‚Ę40“‘OŒć‚Ģ5 ”N–³Ä”­¶‘¶‚Ŗ“¾‚ē‚ź‚é‚ŖCŽķX‚Ģ—\ŒćˆöŽq‚É‚ę‚č—\Œć—ĒDŒQC’†ŠŌŒQC•s—ĒŒQ‚Ģ3 Žķ—Ž‚É‹ę•Ź‚³‚ź‚éB

AML ‚Ģ—\Œć‚É‚ĶŠ³ŽŅ‘¤—vˆö‚Ę”’ŒŒ•a×–E‘¤—vˆö‚Ģ‘o•ū‚ŖŠÖŒW‚·‚é‚Ę‚Ę‚ą‚ɁCŽ”—Ć”½‰ž«‚ą’·Šś—\Œć‚ɉe‹æ‚š‹y‚Ś‚·ˆöŽq‚Ę‚Č‚éi•\3j2j5j`7jB

Š³ŽŅ‘¤—vˆö‚Ę‚µ‚āC”N—īi60 ĪˆČćjC‘Sgó‘ŌiPerformance StatusFPS 3 ‚Ø‚ę‚Ń4jC‡•¹Ē‚Ģ‘¶ŻiŠ“õĒ‚Č‚Ēj‚Ŗ—\Œć•s—ĒˆöŽq‚Ę‚µ‚ďd—v‚Å‚ ‚éB

”’ŒŒ•a×–E‘¤—vˆö‚Ę‚µ‚āCõF‘ĢŠjŒ^C”­Ē—lŽ®ide novo ‚Ü‚½‚Ķ“ńŽŸ«jC‰fŽž”’ŒŒ‹…”C×–EŒ`‘ŌiˆŁŒ`¬‚Ģ—L–³CFAB •aŒ^CmyeloperoxidaseFMPO õF—z«—¦j‚Ŗ—\ŒćˆöŽq‚Ę‚Č‚éB

‹ß”NCõF‘ĢˆŁķ‚Ģ‚Ż‚Č‚ē‚øCŽķX‚Ģˆā“`Žq•ĻˆŁ‚Ŗ—\ŒćˆöŽq‚Ę‚µ‚ďd—v‚Å‚ ‚邱‚Ę‚Ŗ•ń‚³‚ź‚Ä‚Ø‚čC“Į‚ÉAML ‚Ģ–ń1/4 ‚É”F‚ß‚ē‚ź‚鐳ķõF‘ĢŠjŒ^i—\Œć’†ŠŌŒQj‚Ģ—\Œć‚š×•Ŗ‰»‚·‚éˆöŽq‚Ę‚µ‚Ä’–Ś‚³‚ź‚Ä‚¢‚éBNPM1 ˆā“`Žq•ĻˆŁ—z«—į‚ĶŠ°‰š“±“ü—¦‚É‘Ī‚µ‚Ä—ĒD‚ČˆöŽq‚Å‚ ‚éBCEBPA ˆā“`Žq•ĻˆŁ‚ĶŠ°‰š“±“ü‚Ę’·Šś—\Œć‚É‘Ī‚·‚é—ĒD‚ČˆöŽq‚Ę‚µ‚Ä’m‚ē‚ź‚éBˆź•ūCFLT3-ITD ˆā“`Žq•ĻˆŁ‚Ķ’·Šś—\Œć‚É‘Ī‚·‚é•s—ĒˆöŽq‚Ę‚µ‚ďd—v‚Å‚ ‚éB‚±‚ź‚ēˆā“`Žq•ĻˆŁ‚ʏ]—ˆ‚ĢõF‘ĢŠjŒ^‚ÉŠī‚Ć‚­—\ŒćˆöŽq‚š‘g‚Ż‡‚ķ‚¹‚½CV‚½‚Č—\Œć‘w•Ź‰»ƒVƒXƒeƒ€‚ŖEuropean LeukemiaNet ‚ę‚č’ń„‚³‚ź‚Ä‚¢‚éi•\4j8jB

•\3@AML ‚É‚Ø‚Æ‚é—\Œć‘w•Ź‰»ˆöŽq2j5j`7j
‘w•Ź‰»ˆöŽq —ĒD‚Ę‚Č‚éˆöŽq •s—Ē‚Ę‚Č‚éˆöŽq
”N—ī 50 ĪˆČ‰ŗ 60 ĪˆČć
‘Sgó‘ŌiPSj PS 2 ˆČ‰ŗ PS 3 ˆČć
”­Ē—lŽ® de novo “ńŽŸ«
õF‘ĢŠjŒ^ t (8;21)(q22;q22)
inv (16)(p13.1q22)
t (16;16)(P13.1;q22)
t (15;17)(q22;q21)
3qˆŁķminv (3)(q21q26.2)
t (3;3)(q21;q26.2)‚Č‚Ēn
5 ”ŌE7 ”ŌõF‘Ģ‚ĢŒ‡Žø‚Ü‚½‚Ķ’·˜qŒ‡Žø
t (6;9)(p23;q24)•”ŽGŠjŒ^
ˆā“`Žq•ĻˆŁ NPM1 •ĻˆŁ
CEBPA •ĻˆŁ
FLT3-ITD •ĻˆŁ
Š°‰š‚Ü‚Å‚É—v‚µ‚½Ž”—Ɖń” 1 ‰ń 2 ‰ńˆČć
•\4@European LeukemiaNet ‚É‚ę‚é•ŖŽqˆŁķ‚ÉŠī‚Ć‚­‘w•Ź‰»8j
ELN Genetic Risk Group Subsets
Favorable
  • t (8;21)(q22;q22); RUNX1-RUNX1T1
  • inv (16)(p13.1q22) or t (16;16)(p13.1;q22); CBFB-MYH11
  • Mutated NPM1 without FLT3-ITDinormal karyotypej
  • Mutated CEBPAinormal karyotypej
Intermediate-I
  • Mutated NPM1 and FLT3-ITDinormal karyotypej
  • Wt-NPM1 and FLT3-ITDinormal karyotypej
  • Wt-NPM1 without FLT3-ITDinormal karyotypej
Intermediate-II
  • t (9;11)(p22;q23); MLLT3-MLL
  • Cytogenetic abnormalities not classified as favorable or adverse
Adverse
  • inv (3)(q21q26.2) or t (3;3)(q21;q26.2); RPN1-EVI1
  • t (6;9)(p23;q34); DEK-NUP214
  • t (v;11)(v;q23); MLL rearranged
  • -5 or del(5q)
  • -7
  • abnl(17p)
  • complex karyotype
yŽQl•¶Œ£z

1j Ohtake S, et al. Randomized study of induction therapy comparing standard-dose idarubicin with highdose daunorubicin in adult patients with previously untreated acute myeloid leukemia : the JALSG AML201 Study. Blood. 2011 ; 117i8j : 2358-65.i1iiDivj

2j NCCN Clinical Practice Guidelines in Oncology. Acute Myeloid Leukemia. Version 2. 2012.iƒKƒCƒhƒ‰ƒCƒ“j

3j Swerdlow SH, et al. eds. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon, IARC ; 2008.

4j Bennett JM, et al. Proposed revised criteria for the Classification of acute myeloid leukemia. A report of the French-American-British Cooperative Group. Ann Intern Med. 1985 ; 103i4j : 620-5.

5j Grimwade D, et al. The importance of diagnostic cytogenetics on outcome in AML : analysis of 1,612 patients entered into the MRC AML 10 trial. The Medical Research Council Adult and Childrenfs Leukaemia Working Parties. Blood. 1998 ; 92i7j : 2322-33.i3iiiDj

6j “ś–{ŒŒ‰tŠw‰ļD“ś–{ƒŠƒ“ƒp–Ō“ąŒnŠw‰ļ•ŅD‘¢ŒŒŠķŽīį‡Žęˆµ‚¢‹K–ń@‘ę1 ”ŁD2010 ”N3 ŒŽDiƒKƒCƒhƒ‰ƒCƒ“j

7j Kuriyama K, et al. Trial to extract prognostic factors prior to the start of induction chemotherapy for adult AML. Berlin : Springer. 1998 ; pp901-5.

8j Dohner H, et al. Diagnosis and management of acute myeloid leukemia in adults : recommendations from an international expert panel, on behalf of the European LeukemiaNet. Blood. 2010 ; 115i3j : 453-74.iƒŒƒrƒ…[j



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‹}«œ‘«”’ŒŒ•a‚ĢƒAƒ‹ƒSƒŠƒYƒ€ Žį”NŽŅ AML CQ2 CQ5 CQ6 CQ7 CQ8 CQ9

1DŽį”NŽŅAML

AML ‚ʐf’f‚³‚ź‚½ź‡‚Ķć‹L‚ĢƒAƒ‹ƒSƒŠƒYƒ€‚ɏ]‚¤‚±‚Ę‚Ŗ„§‚³‚ź‚éBŽį”NAML ‚É‘Ī‚·‚é•W€“IŠ°‰š“±“ü—Ć–@‚ĶƒAƒ“ƒgƒ‰ƒTƒCƒNƒŠƒ“{•W€—ŹƒVƒ^ƒ‰ƒrƒ“iAraCjiCQ2j‚Å‚ ‚éB‚»‚ĢŪ‚ĢƒAƒ“ƒgƒ‰ƒTƒCƒNƒŠƒ“Œn–ņÜ‚ĢŽŠ“K‚ČŽķ—Ž‚Ę“Š—^—Ź‚Ķˆź‚Ā‚ÉŒĄ’č‚³‚ź‚Č‚¢‚ŖC‚—p—Źƒ_ƒEƒmƒ‹ƒrƒVƒ“iDNRj‚Ü‚½‚ĶƒCƒ_ƒ‹ƒrƒVƒ“iIDRjiķ—p—Źj‚ĢŽg—p‚Ŗ„§‚³‚ź‚éiCQ2jB1 ƒR[ƒX–Ś‚ĢŠ°‰š“±“ü—Ć–@‚Å”ńŠ°‰šĒ—į‚É‘Ī‚µ‚Ä‚Ķ“ƈźƒŒƒWƒƒ“‚ŖŒJ‚č•Ō‚³‚ź‚邱‚Ę‚Ŗ‘½‚­iCQ5jC2 ƒR[ƒX–Ś‚ĢŽ”—Ć‚Å‚ąŠ°‰š‚Ŗ“¾‚ē‚ź‚Č‚¢ź‡‚ĶC‘å—Ź‚ ‚é‚¢‚Ķ’†“™—ŹArac ‚šŠÜ‚Ž‹~‰‡—Ć–@‚Ŗs‚ķ‚ź‚éB

’nŒÅ‚ß—Ć–@‚ĶõF‘Ģ‚Č‚Ē‚Ģ—\ŒćˆöŽq‚É‚ę‚č‘w•Ź‰»‚µ‚čs‚ķ‚ź‚éB—\Œć—ĒDŒQ‚É‘Ī‚µ‚Ä‚ĶCAraC ‘å—Ź—Ć–@iCQ6, 7j‚ŖC—\Œć’†ŠŌŒQC•s—ĒŒQ‚É‘Ī‚µ‚Ä‚Ķ“ÆŽķ‘¢ŒŒŠ²×–EˆŚA‚Ŗ„§‚³‚ź‚é‚ŖiCQ9jC“KŲ‚Čƒhƒi[‚Ŗ•sŻ‚Ģź‡‚ĶC”ńŒš·‘Ļ«‚ĢƒAƒ“ƒgƒ‰ƒTƒCƒNƒŠƒ“Œn–ņÜ‚šŠÜ‚ń‚¾ƒŒƒWƒƒ“‚ŖŽĄŽ{‚³‚ź‚éiCQ8jBˆŪŽ—Ć–@‚Ģ—L—p«‚Ķ–¾‚ē‚©‚Å‚Ķ‚Č‚¢B

‹}«œ‘«”’ŒŒ•a‚ĢƒAƒ‹ƒSƒŠƒYƒ€ ‚—īŽŅ AML CQ4 CQ10

2D‚—īŽŅAML

‚—īŽŅ‚Ģ’č‹`‚Ķ’č‚©‚Å‚Ķ‚Č‚¢‚ŖC‚ķ‚Ŗ‘‚Å‚Ķ65 ĪˆČć‚Ę‚·‚邱‚Ę‚Ŗ‘½‚¢B•W€“I‚ČŽ”—Ć‚Ŗ‰Ā”\‚©‚Ē‚¤‚©‚ĶCPS ‚ā‡•¹ĒC‚³‚ē‚É‚ĶõF‘Ģ•ŖĶ‚ĢŒ‹‰Ź‚šŽQl‚É’S“–ˆćŽt‚É‚ę‚č”»’f‚³‚ź‚éBŽ”—ƉĀ”\‚Ę”»’f‚³‚ź‚½Ē—į‚É‘Ī‚µ‚Ä‚ĶDNR{AraC ‚Ü‚½‚ĶDNR{ƒGƒmƒVƒ^ƒrƒ“iBHACj‚Ŗ„§‚³‚ź‚éiCQ4jB‚—īŽŅAML ‚É‘Ī‚·‚é•W€“IŠ°‰šŒć—Ć–@‚ĶŠm—§‚³‚ź‚Ä‚Č‚¢‚ŖC‚ķ‚Ŗ‘‚Å‚ĶC”ńŒš·‘Ļ«‚ĢƒAƒ“ƒgƒ‰ƒTƒCƒNƒŠƒ“Œn–ņÜ‚šŠÜ‚Ž‘½Ü•¹—pƒŒƒWƒƒ“‚ŖŽĄŽ{‚³‚ź‚邱‚Ę‚Ŗ‘½‚¢BÅ‹ß‚Å‚ĶC—\Œć•s—Ē‚ĢĒ—į‚É‚ĶŠ°‰šŒć—Ć–@‚Ę‚µ‚čœ‘”ń”j‰ó“I‘Oˆ’u‚É‚ę‚é“ÆŽķ‘¢ŒŒŠ²×–EˆŚA‚ąs‚ķ‚ź‚邱‚Ę‚Ŗ‚ ‚éB•W€Ž”—Ć‚Ķ¢“ļ‚¾‚ŖCŽ”—Ć‚Ķ‰Ā”\‚Ę”»’f‚³‚ź‚½Ē—į‚É‚ĶC’į—p—ŹAraC ‚āV‹K–ņÜ‚É‚ę‚鎔—Ć‚Ŗs‚ķ‚ź‚éB



CQ1

AML‚Ģf’fŽž‚É•K—v‚Čˆā“`ŽqŒŸø‚Ķ‰½‚ŖŠ©‚ß‚ē‚ź‚é‚©


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ƒJƒeƒSƒŠ[2A
õF‘ĢŠjŒ^ŒŸø‚Ķ•aŒ^•Ŗ—ŽC—\Œć—\‘ŖCŽ”—Ć–@‘I‘š‚É‚Ø‚¢‚Ä•K{‚Å‚ ‚éBWHO •Ŗ—Ž‚Å‚ĶõF‘Ģ³ķŠjŒ^‚ĢĒ—į‚É‚Ø‚¢‚Ä‚ĶFLT3CNPM1CCEBPA ˆā“`Žq•ĻˆŁ‚Ģ‰šĶ‚šs‚¤‚±‚Ę‚Ŗ„§‚³‚ź‚Ä‚¢‚éB

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AML ×–E‚ĢõF‘ĢŠjŒ^‚ĶCŠ°‰š“±“ü—Ć–@‚É‘Ī‚·‚锽‰ž«‚Ø‚ę‚ѐ¶‘¶‚É‘Ī‚·‚éÅ‚ą‹­‚¢—\ŒćˆöŽq‚Å‚ ‚čCWHO •Ŗ—Ž‚É‚Ø‚Æ‚é•aŒ^f’fC‚³‚ē‚É‚ĶŽ”—Ć–@‚Ģ‘I‘š‚É‚Ø‚¢‚Ä‚ąd—v‚ȏī•ń‚Ę‚Č‚éB

Žį”N¬l‚É‚Ø‚¢‚Ä‚ĶCõF‘ĢŠjŒ^‚ÉŠī‚Ć‚«C—\Œć—ĒDŒQC’†ŠŌŒQC•s—ĒŒQ‚Ģ3 ŒQ‚É•Ŗ—Ž‚³‚ź‚é1j2jBNCCN ƒKƒCƒhƒ‰ƒCƒ“‚Å‚ĶCt (8;21)(q22;q22)Cinv (16)(p13.1q22) ‚Ü‚½‚Ķt (16;16)(p13.1;q22) ‚Ŗ—\Œć—ĒD‚ȐõF‘ĢŠjŒ^Cinv (3)(q21q26.2) ‚Ü‚½‚Ķt (3;3)(q21;q26.2)C-5 ‚Ü‚½‚Ķ del (5q)C-7 ‚Ü‚½‚Ķdel (7q)Ct (6;9)(p23;q34)C•”ŽGŠjŒ^‚Ŗ—\Œć•s—ĒõF‘ĢŠjŒ^‚Ę‚³‚źC‚»‚źˆČŠO‚ĢŠjŒ^‚Ķ—\Œć’†ŠŌŒQ‚É•Ŗ—Ž‚³‚ź‚éBMLL ˆā“`Žqi11q23j‚šŠÜ‚ŽõF‘Ģ“]Ą‚Ķ—\Œć•s—ĒŒQ‚Ę‚³‚ź‚Ä‚¢‚½‚ŖCt (9;11)(p22;q23) ‚É‚Ā‚¢‚Ä‚Ķ—\Œć’†ŠŌŒQ‚É•Ŗ—Ž‚³‚ź‚éBMRC AML-11 ŽŽŒ±‚ĢŒ‹‰Ź‚É‚ę‚é‚ʁC‚—īŽŅAML ‚É‚Ø‚¢‚Ä‚ą‚±‚ź‚ē‚ĢõF‘ĢŠjŒ^‚ÉŠī‚Ć‚­‘w•Ź‰»‚ŖŠY“–‚·‚邱‚Ę‚ŖŽ¦‚³‚ź‚Ä‚¢‚é3jB

ˆź•ūC—\Œć—ĒDŒQ‚Ę‚µ‚Ä•Ŗ—Ž‚³‚ź‚Ä‚¢‚ét (8;21)(q22;q22)Cinv (16)(p13.1q22) ‚Ü‚½‚Ķt (16;16)(p13.1;q22) ŠjŒ^‚š—L‚·‚éĒ—į‚É‚Ø‚¢‚Ä‚ąCKITCFLT3 ˆā“`Žq•ĻˆŁ‚š•¹‚¹Ž‚ĀĒ—į‚Ķ—\Œć•s—Ē‚Å‚ ‚é‰Ā”\«‚Ŗ‚ ‚邱‚Ę‚āCõF‘Ģ³ķŠjŒ^‚š’†S‚Ę‚·‚é—\Œć’†ŠŌŒQ‚É‚Ø‚¢‚Ä‚ĶCFLT3CNPM1CCEBPA ˆā“`Žq‚Č‚Ē‚š‚Ķ‚¶‚ß‚Ę‚·‚鑽‚­‚Ģˆā“`Žq•ĻˆŁ‚Ģ‘¶Ż‚Ģ—L–³‚É‚ę‚čC‚»‚Ģ’·Šś—\Œć‚ŖˆŁ‚Č‚é‰Ā”\«‚ŖŽ¦“‚³‚ź‚Ä‚¢‚é4jBWHO •Ŗ—Ži2008j‚É‚Ø‚¢‚Ä‚ąNPM1CCEBPA ˆā“`Žq•ĻˆŁ‚š—L‚·‚éĒ—į‚ĶAML with recurrent genetic abnormalities ’†‚ĢŽb’č“I‚Čˆź•aŒ^iprovisional entityj‚Ę‚µ‚Ä‹LŚ‚³‚ź‚Ä‚¢‚é‚Ę‚Ę‚ą‚ɁCõF‘Ģ³ķŠjŒ^‚ĢĒ—į‚É‚Ø‚¢‚Ä‚ĶFLT3CNPM1CCEBPA ˆā“`Žq•ĻˆŁ‚Ģ‰šĶ‚šs‚¤‚±‚Ę‚Ŗ„§‚³‚ź‚Ä‚¢‚é5jB

–{–M‚É‚Ø‚¢‚Ä‚Ķ‚±‚ź‚ēˆā“`Žq•ĻˆŁŒŸõ‚Ģ•ŪŒÆ“K—p‚Ŗ“¾‚ē‚ź‚Ä‚Ø‚ē‚øC‚Ü‚½‘½‚­‚Ģˆā“`Žq•ĻˆŁ‰šĶ‚ĶŒ¤‹†ŽŗƒŒƒxƒ‹‚Å‚Ģ‚ŻŽĄŽ{‰Ā”\‚Å‚ ‚éB

European LeukemiaNet ‚ĶCFLT3CNPM1CCEBPA ˆā“`Žq•ĻˆŁ‚Ģ‰šĶ‚Ķ—Տ°ŽŽŒ±‚É‚Ø‚¢‚Ä‚Ķs‚¤‚ׂ«‚Å‚ ‚é‚ŖC“śķ—Տ°‚É‚Ø‚¢‚Ä‚ĶCõF‘Ģ³ķŠjŒ^‚Ģź‡‚ɉšĶ‚š„§‚·‚é‚Ę‚µ‚Ä‚¢‚é6jB

yŽQl•¶Œ£z

1j Grimwade D, et al. The importance of diagnostic cytogenetics on outcome in AML : analysis of 1,612 patients entered into the MRC AML 10 trial. The Medical Research Council Adult and Childrenfs Leukaemia Working Parties. Blood. 1998 ; 92i7j : 2322-33.i3iiiDj

2j Slovak ML, et al. Karyotypic analysis predicts outcome of preremission and postremission therapy in adult acute myeloid leukemia : a Southwest Oncology Group/Eastern Cooperative Oncology Group Study. Blood. 2000 ; 96i13j : 4075-83.i3iiiDj

3j Grimwade D, et al. The predictive value of hierarchical cytogenetic classification in older adults with acute myeloid leukemia iAMLj : analysis of 1065 patients entered into the United Kingdom Medical Research Council AML11 trial. Blood. 2001 ; 98i5j : 1312-20.i3iiiDj

4j Marcucci G, et al. Molecular genetics of adult acute myeloid leukemia : prognostic and therapeutic implications. J Clin Oncol. 2011 ; 29i5j : 475-86.iƒŒƒrƒ…[j

5j Swerdlow SH, et al. eds. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon, IARC ; 2008.

6j Döhner H, et al. Diagnosis and management of acute myeloid leukemia in adults : recommendations from an international expert panel, on behalf of the European LeukemiaNet. Blood. 2010 ; 115i3j : 453-74.iƒŒƒrƒ…[j


CQ2

Žį”NŽŅde novo AML ‚É‘Ī‚·‚é•W€“IŠ°‰š“±“ü—Ć–@‚Ę‚µ‚Ä‚Ē‚ĢƒŒƒWƒƒ“‚ŖŠ©‚ß‚ē‚ź‚é‚©


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Žį”N¬lde novo AML ‚É‘Ī‚·‚é•W€“IŠ°‰š“±“ü—Ć–@‚ĶƒAƒ“ƒgƒ‰ƒTƒCƒNƒŠƒ“iƒCƒ_ƒ‹ƒrƒVƒ“‚Ü‚½‚Ķ‚—p—Źƒ_ƒEƒmƒ‹ƒrƒVƒ“j{ •W€—ŹƒVƒ^ƒ‰ƒrƒ“‚Å‚ ‚éB

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]—ˆC60 Ī–¢–ž‚ĢŽį”N¬lde novo AML ‚É‘Ī‚·‚é•W€“IŠ°‰š“±“ü—Ć–@‚ĶCƒ_ƒEƒmƒ‹ƒrƒVƒ“iDNRj45`60 mg/m2 3 “śŠŌ{ƒVƒ^ƒ‰ƒrƒ“iAraCj100 mg/m2 ‚Ü‚½‚Ķ200 mg/m2 7 “śŠŌŽ‘±“Š—^‚Ģg3{7h—Ć–@‚Å‚ ‚Į‚½‚ŖCƒCƒ_ƒ‹ƒrƒVƒ“iIDRj{AraC ‚ĘDNR{AraC ‚Ę‚Ģ”äŠrŽŽŒ±‚Ø‚ę‚Ńƒƒ^ƒAƒiƒŠƒVƒX‚ĢŒ‹‰ŹCIDR{AraC ‚ĢDNR{AraC ‚É‘Ī‚·‚é—D‰z«‚Ŗ•ń‚³‚ź‚½1jB‚µ‚©‚µC]—ˆ‚ĢDNR “Š—^—Źi45`60 mg/m2j‚ĶIDR “Š—^—Źi12 mg/m2j‚Ę”äŠr‚µ‚āC¶•ØŠw“I‚ɏ­‚Č‚¢‚±‚Ę‚ŖŽw“E‚³‚ź‚½B

Eastern Cooperative Oncology GroupiECOGj‚Å‚ĶC60 Ī–¢–ž‚Ģde novo AML ‚É‘Ī‚µ‘—ŹDNRi90 mg/m2j3 “śŠŌ{AraCi100 mg/m2j7 “śŠŌ‚ʏ]—ˆ‚ĢDNRi45 mg/m2j3 “śŠŌ{AraCi100 mg/m2j7 “śŠŌ‚Ę‚Ģƒ‰ƒ“ƒ_ƒ€‰»”äŠrŽŽŒ±‚šŽĄŽ{‚µCŠ°‰š—¦C¶‘¶Š„‡‚Ę‚ą‚ɍ‚—p—ŹDNRi90 mg/m2jŒQ‚Ŗ—LˆÓ‚É—D‚ź‚Ä‚¢‚½‚±‚Ę‚©‚ēCDNR{AraCg3{7h—Ć–@‚É‚Ø‚Æ‚éDNR ŽŠ“K“Š—^—Ź‚Ķ90 mg/m2 3 “śŠŌ‚Å‚ ‚邱‚Ę‚ŖŽ¦‚³‚ź‚½2jB

DNRi90 mg/m2j{AraC ‚ĘIDR{AraC ‚Ę‚Ģ”äŠrŽŽŒ±‚Ķs‚ķ‚ź‚Ä‚¢‚Č‚¢‚ŖCJapan Adult Leukemia Study GroupiJALSGj‚ÅŽĄŽ{‚³‚ź‚½DNRi50 mg/m2j5 “śŠŌ{AraC ‚ĘIDR{AraC ‚Ę‚Ģƒ‰ƒ“ƒ_ƒ€‰»”äŠrŽŽŒ±iAML 201 stydyj‚ĢŒ‹‰Ź‚Å‚ĶCŠ°‰š—¦‚Ø‚ę‚ѐ¶‘¶Š„‡‚Ę‚ą‚É“Æ“™«‚ŖŽ¦‚³‚ź‚Ä‚¢‚é3jB

‚»‚Ģ‘¼‚ĢƒAƒ“ƒgƒ‰ƒTƒCƒNƒŠƒ“Œn–ņÜ‚Ę‚µ‚āCƒ~ƒgƒLƒTƒ“ƒgƒƒ“iMITji12 mg/m2j‚ĘDNRi45 mg/m2j‚Ę‚Ģ”äŠrŽŽŒ±‚Ŗs‚ķ‚ź‚Ä‚¢‚é‚ŖC¶‘¶Š„‡‚É—LˆÓ·‚Ķ”F‚ß‚ē‚ź‚Ä‚¢‚Č‚¢4jB

‚µ‚½‚Ŗ‚Į‚āCŽį”N¬lde novo AML ‚É‘Ī‚·‚é•W€“IŠ°‰š“±“ü—Ć–@‚ĶCIDR{AraC ‚Ü‚½‚Ķ‚—p—ŹDNR{AraC ‚Å‚ ‚éB‚½‚¾‚µC‚—p—ŹDNR ‚Ģ“Š—^–@‚Ę‚µ‚āCDNRi90 mg/m2j3 “śŠŌ‚ĘDNRi50 mg/m2j5 “śŠŌ‚Ģ”äŠrŽŽŒ±‚ĶŽĄŽ{‚³‚ź‚Ä‚¢‚Č‚¢B

yŽQl•¶Œ£z

1j A systematic collaborative overview of randomized trials comparing idarubicin with daunorubicin ior other anthracyclinesj as induction therapy for acute myeloid leukaemia. The AML Collaborative Group. Br J Haematol. 1998 ; 103i1j : 100-9.i1iiAj

2j Fernandez HF, et al. Anthracycline dose intensification in acute myeloid leukemia. N Engl J Med. 2009 ; 361i13j : 1249-59.i1iiDivj

3j Ohtake S, et al. Randomized study of induction therapy comparing standard-dose idarubicin with highdose daunorubicin in adult patients with previously untreated acute myeloid leukemia : the JALSG AML201 Study. Blood. 2011 ; 117i8j : 2358-65.i1iiDivj

4j Arlin Z, et al. Randomized multicenter trial of cytosine arabinoside with mitoxantrone or daunorubicin in previously untreated adult patients with acute nonlymphocytic leukemia iANLLj. Lederle Cooperative Group. Leukemia. 1990 ; 4i3j : 177-83.i1iiAj


CQ3

Žį”NŽŅde novo AML ‚ĢŠ°‰š“±“ü—Ć–@iƒAƒ“ƒgƒ‰ƒTƒCƒNƒŠƒ“{•W€—ŹƒVƒ^ƒ‰ƒrƒ“j‚É‘¼‚Ģ–ņÜ‚Ģ’Ē‰Į‚āƒVƒ^ƒ‰ƒrƒ“‘å—Ź—Ć–@‚Ģ‘g‚Żž‚Ż‚Ķ—LŒų‚©


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•W€“IŠ°‰š“±“ü—Ć–@‚Å‚ ‚éƒAƒ“ƒgƒ‰ƒTƒCƒNƒŠƒ“iƒCƒ_ƒ‹ƒrƒVƒ“‚Ü‚½‚Ķƒ_ƒEƒmƒ‹ƒrƒVƒ“j{•W€—ŹƒVƒ^ƒ‰ƒrƒ“—Ć–@‚É‘¼Ü‚š’Ē‰Į‚µ‚½ź‡‚Ģ—D‰z«‚Ķ”F‚ß‚ē‚ź‚Ä‚¢‚Č‚¢B‚Ü‚½CƒVƒ^ƒ‰ƒrƒ“‘å—Ź—Ć–@‚š‘g‚Ż“ü‚ź‚½ź‡‚Ģ—D‰z«‚ĢƒGƒrƒfƒ“ƒX‚Ķ–R‚µ‚­C—LŠQŽ–Ū‚ĢŠėŒÆ«‚Ŗ‘‚·‚½‚ߐ„§‚³‚ź‚Č‚¢B

y‰š ąz

ƒAƒ“ƒgƒ‰ƒTƒCƒNƒŠƒ“mƒCƒ_ƒ‹ƒrƒVƒ“iIDRj‚Ü‚½‚Ķƒ_ƒEƒmƒ‹ƒrƒVƒ“iDNRjn3 “śŠŌ{•W€—ŹƒVƒ^ƒ‰ƒrƒ“iAraCj7 “śŠŌ‚É‚ę‚銰‰š“±“ü—Ć–@‚Ƀ`ƒIƒOƒAƒjƒ“‚āƒGƒgƒ|ƒVƒhiETPj‚š‰Į‚¦‚½ź‡‚Ģ—D‰z«‚ÉŠÖ‚·‚éƒGƒrƒfƒ“ƒX‚Ķ–R‚µ‚¢BAustralian Leukemia Study GroupiALSGj‚Å‚ĶCDNRi50 mg/m2j3 “śŠŌ{AraCi100 mg/m2j7 “śŠŌ‚ĘDNRi50 mg/m2j3 “śŠŌ{AraCi100 mg/m2j 7 “śŠŌ{ETPi75 mg/m2j7 “śŠŌ‚Ę‚Ģƒ‰ƒ“ƒ_ƒ€‰»”äŠrŽŽŒ±‚Ŗs‚ķ‚ź‚½BŠ°‰šŠśŠŌ’†‰›’l‚ĶƒGƒgƒ|ƒVƒh’Ē‰ĮŒQ‚Å—LˆÓ‚É’·Šś‚Å‚ ‚Į‚½i18 ƒJŒŽvs 12 ƒJŒŽj‚ŖCŠ°‰š—¦C¶‘¶Š„‡‚Å‚Ķ—¼ŒQŠŌ‚É—LˆÓ·‚š”F‚ß‚Ä‚¢‚Č‚¢1jB

Š°‰š“±“ü—Ć–@‚É‚Ø‚Æ‚éAraC ‘å—Ź—Ć–@iHiDACj‚ĢˆÓ‹`‚É‚Ā‚¢‚Ä‚ĶCALSG ‚ĘSouthwestern Oncology GroupiSWOGj‚щƒ“ƒ_ƒ€‰»”äŠrŽŽŒ±‚ŖŽĄŽ{‚³‚ź‚Ä‚¢‚éB

ALSG ‚Å‚ĶDNRi50 mg/m2j3 “śŠŌ{ETPi75 mg/m2j7 “śŠŌ{AraCi100 mg/m2j7 “śŠŌ‚ĘDNRi50 mg/m2j3 “śŠŌ{ETPi75 mg/m2j7 “śŠŌ{AraCi3 g/m2j12 ŽžŠŌ–ˆ4 “śŠŌ‚Ģƒ‰ƒ“ƒ_ƒ€‰»”äŠrŽŽŒ±‚ŖŽĄŽ{‚³‚ź‚½B5 ”N–³Ä”­¶‘¶Š„‡iRFSj‚ĶHiDAC ŒQ‚Ŗ—LˆÓ‚É—D‚ź‚Ä‚¢‚½i48“ vs 25“j‚ŖC¶‘¶Š„‡CŠ°‰š—¦‚Å‚Ķ—LˆÓ·‚š”F‚ß‚Ä‚¢‚Č‚¢2jB

SWOG ‚Å‚ĶDNRi45 mg/m2j3 “śŠŌ{AraCi200 mg/m2j7 “śŠŌ‚ĘDNRi45 mg/m2j3 “śŠŌ{AraCi2 g/m2j12 ŽžŠŌ–ˆ6 “śŠŌ‚Ģƒ‰ƒ“ƒ_ƒ€‰»”äŠrŽŽŒ±‚ŖŽĄŽ{‚³‚ź‚½B4 ”NRFS ‚ĶHiDAC ŒQ‚Ŗ—D‚ź‚Ä‚¢‚½i33“ vs 21“Cp0.049j‚ŖC¶‘¶Š„‡CŠ°‰š—¦‚Å‚Ķ—LˆÓ·‚š”F‚ß‚Ä‚¢‚Č‚¢B‚Ü‚½CHiDAC ŒQ‚Å‚Ķ—LˆÓ‚ÉŽ”—ĆŠÖ˜AŽ€–SiTRMj‚ʐ_Œo“Ő«‚Ŗ‚•p“x‚É”F‚ß‚ē‚ź‚Ä‚¢‚é3jB

HiDAC ‚ʍ‚—p—ŹDNR ‚Ü‚½‚ĶIDR •¹—p—Ć–@‚ÉŠÖ‚·‚éƒGƒrƒfƒ“ƒX‚Ķ‚Č‚¢B

•W€“IŠ°‰š“±“ü—Ć–@‚Å‚ ‚éƒAƒ“ƒgƒ‰ƒTƒCƒNƒŠƒ“iDNR ‚Ü‚½‚ĶIDRj{•W€—ŹAraC —Ć–@‚É‘¼Ü‚Ģ’Ē‰Į‚µ‚½ź‡C‚Ø‚ę‚ŃHiDAC —Ć–@‚š‘g‚Ż“ü‚ź‚½ź‡‚Ģ—D‰z«‚ÉŠÖ‚·‚éƒGƒrƒfƒ“ƒX‚Ķ–R‚µ‚¢B‚Ü‚½CHiDAC ‚š‘g‚Ż“ü‚ź‚½ź‡‚É‚ĶTRMC_Œo“Ő«‚Č‚Ē‚Ģ—LŠQŽ–Ū‚ĢŠėŒÆ«‚Ŗ‘‚·‚½‚߁CHiDAC ‚Ģ‘g‚Ż“ü‚ź‚Ķ„§‚³‚ź‚Č‚¢B

yŽQl•¶Œ£z

1j Bishop JF, et al. Etoposide in acute nonlymphocytic leukemia. Australian Leukemia Study Group. Blood. 1990 ; 75i1j : 27-32.i1iiDivj

2j Bishop JF, et al. A randomized study of high-dose cytarabine in induction in acute myeloid leukemia. Blood. 1996 ; 87i5j : 1710-7.i1iiAj

3j Weick JK, et al. A randomized investigation of high-dose versus standard-dose cytosine arabinoside with daunorubicin in patients with previously untreated acute myeloid leukemia : a Southwest Oncology Group study. Blood. 1996 ; 88i8j : 2841-51.i1iiAj


CQ4

‚—īŽŅAML ‚É‘Ī‚µ‚Đ„§‚³‚ź‚銰‰š“±“ü—Ć–@‚Ķ‰½‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2A
60 Ī‚©‚ē65 Ī‚Ü‚Å‚Ģ‚—īŽŅAML ‚É‚Ø‚¢‚Ä‚ĶCŽį”N¬l‚Ę“Æ“™‚ĢŠ°‰š“±“ü—Ć–@‚šŽĄŽ{‚µ‚½•ū‚Ŗ—ĒD‚ČŠ°‰š—¦‚ʐ¶‘¶Š„‡‚ŖŠś‘Ņ‚Å‚«‚éB‚µ‚©‚µC‚—īŽŅAML ‚Å‚Ķ‘Sgó‘ŌiPSjC•¹‘¶Ē‚Č‚Ē‚Ģ’ö“x‚É‚ę‚čCŽ”—Ć‹­“x‚ĢŒyŒø‚ābest supportive care ‚Ģ‘I‘š‚šŒŸ“¢‚·‚邱‚Ę‚Ŗ•K—v‚Å‚ ‚éB

y‰š ąz

‚—īŽŅ‚Ģ’č‹`‚Ķ–¾Šm‚Å‚Ķ‚Č‚¢‚ŖCˆź”Ź“I‚É60 ĪˆČć‚ĢAML Š³ŽŅ‚Å‚ĶC—ļ”N—ī‚¾‚Æ‚Å‚Ķ‚Č‚­C‘Sgó‘Ōiperformans statusFPSj‚ā•¹‘¶Ē‚Č‚ē‚Ń‚ÉAML ‚Ģ“Į«iõF‘ĢŠjŒ^‚ā”­Ē—lŽ®j‚É‚ę‚Į‚ÄŽ”—Ć–@‚Ģ‘I‘š‚šs‚¤‚ׂ«‚Å‚ ‚éB

‚—īŽŅAML ‚É‘Ī‚·‚銰‰š“±“ü—Ć–@‚ÅŽį”NŽŅ‚Ę“Æ—l‚É•W€“IŠ°‰š“±“ü‰»Šw—Ć–@‚šs‚¤ź‡‚ʁC’į—p—Ź‚ĢŽ”—Ć‚Č‚¢‚µbest supportive care ‚š”äŠr‚·‚é‘O•ūŒüŽ‹“IŽŽŒ±‚Å‚ĶC•W€“IŠ°‰š“±“ü—Ć–@ŒQ‚ĶŠ°‰š—¦E¶‘¶Š„‡‚Ę‚ą‚ɐ¬Ń‚ŖŸ‚邱‚Ę‚ŖŽ¦‚³‚ź‚Ä‚¢‚é1jB‚µ‚©‚µC‚—īŽŅAML ‚É‚Ø‚¢‚Ä‚ĶPSC‡•¹Ē‚ŖŽ”—Ɛ¬Ń‚É‹y‚Ś‚·‰e‹æ‚Ŗ‹­‚¢‚±‚Ę‚É—ÆˆÓ‚·‚é•K—v‚Ŗ‚ ‚éB‡•¹Ē‚Ŗ‚Č‚­—ĒD‚Č‘Sgó‘ŌiPS 0`1j‚Å‚ ‚čC—\Œć—ĒD‚ȐõF‘ĢŠjŒ^‚š—L‚·‚鍂—īŽŅAML ‚Å‚ĶC”N—ī‚ÉŠÖŒW‚Č‚­•W€“I‚ČƒAƒ“ƒgƒ‰ƒTƒCƒNƒŠƒ“3 “śŠŌ{•W€—ŹƒVƒ^ƒ‰ƒrƒ“iAraCj7 “śŠŌ‚©‚ē‚Ȃ銰‰š“±“ü—Ć–@‚Ģ‰¶Œb‚šŽó‚Ƃ邱‚Ę‚Ŗ‚Å‚«‚é‰Ā”\«‚Ŗ‚ ‚éB‚µ‚©‚µC75 ĪˆČćC‚ ‚é‚¢‚Ķ60`74 Ī‚Ü‚Å‚ĢŠ³ŽŅ‚Å‚ ‚Į‚Ä‚ąd“Ä‚Č•¹‘¶Ē‚āPS 3 ˆČć‚Ģź‡‚É‚ĶCŽ”—ĆŠÖ˜AŽ€–SiTRMj‚ĢŠėŒÆ«‚Ŗ‚‚¢‚½‚߁C‘¼‚ĢŽ”—Ć‹­“x‚Ģ’į‚¢Ž”—Ć–@‚Ü‚½‚Ķbest supportive care ‚š‘I‘š‚·‚ׂ«‚Å‚ ‚é2jBMRC AML14 ŽŽŒ±‚Å‚ĶC’į—p—ŹAraCi20 mg/m2 ”ē‰ŗ’1 “ś2 ‰ńj—Ć–@‚É‚Ø‚¢‚Ä‚ąC30 “śˆČ“ą‚ĢTRM ‚Ķ26“‚É”F‚ß‚Ä‚¢‚é3jB

Dutch-Belgian Hemato-Oncology Cooperative GroupiHOVONj/Swiss Group for Clinical Cancer ResearchiSAKKj/German AML Study GroupiAMLSGj‚Å‚ĶC60 ĪˆČć‚Ģ‚—īŽŅAML ‚É‘Ī‚µC•W€—Ź‚Ģƒ_ƒEƒmƒ‹ƒrƒVƒ“iDNRji45 mg/m2j{AraC ‚ʍ‚—p—ŹDNRi90 mg/m2j{AraC —Ć–@‚Ģƒ‰ƒ“ƒ_ƒ€‰»”äŠrŽŽŒ±‚ŖŽĄŽ{‚³‚ź‚½B‚—p—ŹDNRi90 mg/m2jŒQ‚Ķ•W€—Ź‚ĢDNR ŒQ‚É”äŠr‚µ‚Ä—LˆÓ‚ɍ‚‚¢Š°‰š—¦‚šŽ¦‚µ‚½‚ŖC¶‘¶Š„‡‚É—LˆÓ·‚š”F‚ß‚Ä‚¢‚Č‚¢B”N—ī•Ź‚ɉšĶ‚µ‚½ź‡C60`65 Ī‚Ü‚Å‚ĢĒ—į‚É‚Ø‚¢‚Ä‚Ģ‚ŻC‚—p—ŹDNRi90 mg/m2jŒQ‚Ķ•W€—Ź‚ĢDNR ŒQ‚É”äŠr‚µ‚Ä—LˆÓ‚ɍ‚‚¢Š°‰š—¦‚ʐ¶‘¶Š„‡‚šŽ¦‚µ‚Ä‚¢‚é4jBJapan Adult Leukemia Study GroupiJALSGj‚É‚Ø‚¢‚Ä‚ĶC65 Ī–¢–ž‚ĢAML ‚Ķ“Æ‚¶Ž”—Ć‹­“x‚Ŗ‘I‘š‚³‚ź‚Ä‚¢‚é‚Ŗd“Ä‚Č—LŠQŽ–Ū‚Ķ•ń‚³‚ź‚Ä‚¢‚Č‚¢5jB

”N—īCPSC‡•¹Ē‚Č‚Ē‚ÉŠī‚Ć‚­‚—īŽŅAML ‚É‘Ī‚·‚鎔—Ć‹­“x‚ĢŒøŽć‹K€‚ÉŠÖ‚µ‚Ä‚Ģ–¾Šm‚ČƒGƒrƒfƒ“ƒX‚Ķ‚Č‚¢BJALSG GML200 ŽŽŒ±‚Å‚ĶC65`69 Ī‚Ü‚Å‚ĢĒ—į‚É‘Ī‚µ‚Ä‚ĶDNRi40 mg/m2j3 “śŠŌ{ƒGƒmƒVƒ^ƒrƒ“iBHACji200 mg/m2j8 “śŠŌC70 ` 79 Ī‚ĢĒ—į‚É‘Ī‚µ‚Ä‚ĶDNRi30 mg/m2j3 “śŠŌ{BHACi200 mg/m2j8 “śŠŌ‚É‚ę‚銰‰š“±“ü—Ć–@‚ŖŽĄŽ{‚³‚ź‚Ä‚Ø‚čC”N—ī‚ÉŠī‚Ć‚­DNR “Š—^—Ź‚Ģ–ŚˆĄ‚É‚Č‚é‚ĘŽv‚ķ‚ź‚é6jB

yŽQl•¶Œ£z

1j Löwenberg B, et al. On the value of intensive remission-induction chemotherapy in elderly patients of 65{years with acute myeloid leukemia : a randomized phase Ⅲ study of the European Organization for Research and Treatment of Cancer Leukemia Group. J Clin Oncol. 1989 ; 7i9j : 1268-74.i1iiAj

2j Döhner H, et al. Diagnosis and management of acute myeloid leukemia in adults : recommendations from an international expert panel, on behalf of the European LeukemiaNet. Blood. 2010 ; 115i3j : 453-74.iƒŒƒrƒ…[j

3j Burnett AK, et al. A comparison of low-dose citarabine and hydroxyurea with or without all-trans retinoic acid for acute myeloid leukemia and high-risk myelodysplastic syndrome in patients not considered fit for intensive treatment. Cancer. 2007 ; 109i6j : 1114-24.i1iiAj

4j Löwenberg B, et al. High-dose daunorubicin in older patients with acute myeloid leukemia. N Engl J Med. 2009 ; 361i13j : 1235-48.i1iiAj

5j Ohtake S, et al. Randomized study of induction therapy comparing standard-dose idarubicin with highdose daunorubicin in adult patients with previously untreated acute myeloid leukemia : the JALSG AML201 Study. Blood. 2011 ; 117i8j : 2358-65.i1iiDivj

6j Wakita A, et al. Randomized comparison of fixed-schedule versus response-oriented individualized induction therapy and use of ubenimex during and after consolidation therapy for elderly patients with acute myeloid leukemia : the JALSG GML200 Study. Int J Hematol. 2012 ; 96i1j : 84-93.i1iiiDivj


CQ5

1 ‰ń‚ĢŠ°‰š“±“ü—Ć–@‚ÅŠ®‘SŠ°‰š‚Ŗ“¾‚ē‚ź‚Č‚¢ź‡C‚Ē‚Ģ‚ꂤ‚ČŽ”—Ć–@‚š‘I‘š‚·‚ׂ«‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2B
“ƈź‚ĢŠ°‰š“±“ü—Ć–@‚š‚ą‚¤ˆź“xŒJ‚č•Ō‚·‚ׂ«‚©CŽ”—Ć–@‚š•Ļ‚¦‚é‚ׂ«‚©‚ĢƒGƒrƒfƒ“ƒX‚Ķ‘¶Ż‚µ‚Č‚¢B‚µ‚©‚µCˆź’č“x‚ĢŠ°‰š‚Ŗ“¾‚ē‚ź‚邱‚Ę‚©‚ēC“ƈź‚ĢŠ°‰š“±“ü—Ć–@‚šÄ“xŒJ‚č•Ō‚·‚±‚Ę‚Ķ‘Ć“–‚ʍl‚¦‚ē‚ź‚éB

y‰š ąz

‚ķ‚Ŗ‘‚É‚Ø‚Æ‚éAML ‚ĢŠ°‰š“±“ü—Ć–@‚ĶJapan Adult Leukemia Study GroupiJALSGj‚ĢƒvƒƒgƒR[ƒ‹‚ōs‚ķ‚ź‚邱‚Ę‚Ŗ‘½‚¢BJALSG ‚Å‚Ķ‚±‚ź‚Ü‚ÅAML87C89C92C95C97C201 ‚ĢŒ¤‹†‚šI—¹‚µCŒ»ŻAML209 Œ¤‹†‚šŽĄŽ{‚µ‚Ä‚¢‚éB‚¢‚ø‚ź‚ĢƒvƒƒgƒR[ƒ‹‚Å‚ą1 ƒR[ƒX–Ś‚ÅŠ°‰š‚µ‚Č‚©‚Į‚½ź‡‚ĶC‚ą‚¤1 ƒR[ƒX“Æ‚¶Ž”—Ć‚šŒJ‚č•Ō‚·‚±‚Ę‚É‚Č‚Į‚Ä‚¢‚éB‚±‚ź‚ē‚Ģ¬Ń‚Å‚Ķ1 ƒR[ƒX‚Å‚ĢŠ°‰š—¦‚Ķ57`72“‚ʍ·‚Ŗ‚ ‚é‚ą‚Ģ‚ĢC‚¢‚ø‚ź‚ĢŒ¤‹†‚Å‚ąŠ°‰š—¦‚Ķ‚Ø‚µ‚ȂׂÄ80“‘OŒć‚Å‚ ‚é1j`5jB1ƒR[ƒX‚ÅŠ°‰š‚µ‚Č‚©‚Į‚½Ē—į‚Å‚Ķ1 ƒR[ƒX–Ś‚ĢR”’ŒŒ•aÜ‚É’ļR«‚Å‚ ‚鏼‡‚Ŗ‘½‚­C“Æ‚¶Ž”—Ć–@‚š—p‚¢‚½ź‡‚Ģ2 ƒR[ƒX–Ś‚ĢŠ°‰š—¦‚Ķ’į‚­CŽ”—Ć–ņ‚š‘悦‚邱‚Ę‚Ģ——R‚É‚Ķ‚Č‚éB‚µ‚©‚µC2 ƒR[ƒX–Ś‚ĢŽ”—Ć‚š‘Ö‚¦‚½‚±‚Ę‚É‚ę‚čCŠ°‰š—¦‚ ‚é‚¢‚Ķ‘S¶‘¶Š„‡iOSj‚ŖŒüć‚·‚é‚Ę‚¢‚¤ƒGƒrƒfƒ“ƒX‚Ķ‚Č‚¢B

yŽQl•¶Œ£z

1j Ohno R, et al. Randomized study of individualized induction therapy with or without vincristine, and of maintenance-intensification therapy between 4 or 12 courses in adult acute myeloid leukemia. AML-87 Study of the Japan Adult Leukemia Study Group. Cancer. 1993 ; 71i12j : 3888-95.i1iiDiv/3iDivj

2j Kobayashi T, et al. Randomized trials between behenoyl cytarabine and cytarabine in combination induction and consolidation therapy, and with or without ubenimex after maintenance/intensification therapy in adult acute myeloid leukemia. The Japan Leukemia Study Group. J Clin Oncol. 1996 ; 14 i1j : 204-13.i1iiDiv/3iDivj

3j Miyawaki S, et al. No benefi cial effect from addition of etoposide to daunorubicin, cytarabine, and 6-mercaptopurine in individualized induction therapy of adult acute myeloid leukemia : the JALSG-AML92 study. Japan Adult Leukemia Study Group. Int J Hematol. 1999 ; 70i2j : 97-104.i1iiDiv/3iDivj

4j Ohtake S, et al. Randomized trial of response-oriented individualized versus fixed-schedule induction chemotherapy with idarubicin and cytarabine in adult acute myeloid leukemia : the JALSG AML95 study. Int J Hematol. 2010 ; 91i2j : 276-83.i1iiDiv/3iDivj

5j Miyawaki S, et al. A randomized, postremission comparison of four courses of standard-dose consolidation therapy without maintenance therapy versus three courses of standard-dose consolidation with maintenance therapy in adults with acute myeloid leukemia : the Japan Adult Leukemia Study Group AML 97 Study. Cancer. 2005 ; 104i12j : 2726-34.i1iiDii/3iDivj


CQ6

ƒVƒ^ƒ‰ƒrƒ“‘å—Ź—Ć–@‚Ķ‚·‚ׂĂĢAML ‚ĢŠ°‰šŒć—Ć–@‚Ę‚µ‚čs‚¤‚ׂ«‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2A
AML ‚ĢŠ°‰šŒć—Ć–@‚Ę‚µ‚Ä‚Ķ60 ĪˆČ‰ŗ‚ĢCBF ”’ŒŒ•a‚É‘Ī‚µ‚ăVƒ^ƒ‰ƒrƒ“‘å—Ź—Ć–@‚Ķ–³•a¶‘¶ŠśŠŌ‚Ģ‰„’·‚ŖŠś‘Ņ‚Å‚«„§‚³‚ź‚éB

y‰š ąz

Cancer and Leukemia Study Group BiCALGBj‚ĶAML Š°‰šŒć—Ć–@‚Ę‚µ‚ăVƒ^ƒ‰ƒrƒ“iAraCj’Źķ—Źi100 mg/m2/dayC5 “śŠŌŽ‘±jC’†“™—Źi400 mg/m2/dayC5 “śŠŌŽ‘±j‚Ø‚ę‚ŃAraC ‘å—Ź—Ć–@i3 g/m2C1 “ś2 ‰ń3 ŽžŠŌ‚ÅĆ’Cday1C3C5 ‚É“Š—^j‚Ģ3 ŒQ‚š‘O•ūŽ‹“I‚É”äŠr‚µ‚½B‚»‚ĢŒ‹‰ŹC60 ĪˆČ‰ŗ‚ÅAraC ‘å—Ź—Ć–@‚Ģ—LŒų«‚ŖŠm”F‚³‚ź‚½1jB

CALGB ‚Ķ‚±‚ĢŒ¤‹†‚Å‚³‚ē‚ɉšĶ‚šs‚¢Ccore binding factoriCBFj”’ŒŒ•a‚Å‚ĶAraC ‘å—Ź—Ć–@‚ŖÅ‚ąŒų‰Ź‚Ŗ‚‚­C³ķŠjŒ^‚Å‚ąŒų‰Ź‚š”F‚ß‚½‚ŖC‚»‚Ģ‘¼‚ĢŠjŒ^‚Å‚ĶŒų‰Ź‚Ŗ–R‚µ‚©‚Į‚½2jB‚Ü‚½CCALGB ‚ĢŒć•ūŽ‹“I‚ȉšĶ‚Å‚ąt (8;21jAML ‚É‘Ī‚µ‚Ä 3 ƒR[ƒXˆČć‚Ģ AraC ‘å—Ź—Ć–@‚Ŗ—LŒų‚Å‚ ‚邱‚Ę‚ŖŽ¦‚³‚ź‚Ä‚¢‚é3jB‚ķ‚Ŗ‘‚ōs‚ķ‚ź‚½‘O•ūŽ‹“IŽŽŒ±‚Å‚ĶC2 g/m2C1 “ś2 ‰ńC5 “ś‚Ę‚±‚ź‚Ü‚Å‚Ģ‘½Ü•¹—p—Ć–@‚Ę”äŠr‚µ‚Ä—¼ŒQ‚É–³•a¶‘¶ŠśŠŌiDFSjC‘S¶‘¶ŠśŠŌiOSj‚Ę‚ą‚ɍ·‚Ŗ‚Č‚©‚Į‚½‚ŖCCBF ”’ŒŒ•a‚Å‚ĶAraC ‘å—Ź—Ć–@ŒQ‚ÅDFS ‚Ģ‰ü‘PŒXŒü‚Ŗ”F‚ß‚ē‚ź‚½4jB‹ß”NCƒhƒCƒc‚ĢƒOƒ‹[ƒv‚©‚ē‚ąAraC 36 g/m2 ‚Ę12 g/m2 ‚Ģ‘O•ūŽ‹“I”äŠrŽŽŒ±‚Ģ¬Ń‚Ŗ•ń‚³‚ź‚½‚ŖC—¼ŒQŠŌ‚ÅDFS/OS ‚ɍ·‚Ŗ‚Č‚­CõF‘Ģ‚É‚ę‚éƒTƒuƒOƒ‹[ƒv‰šĶ‚Å‚ą·‚Ŗ‚Č‚©‚Į‚½5jB

yŽQl•¶Œ£z

1j Mayer RJ, et al. Intensive postremission chemotherapy in adults with acute myeloid leukemia. Cancer and Leukemia Group B. N Engl J Med. 1994 ; 331i14j : 896-903.i1iiAj

2j Bloomfield CD, et al. Frequency of prolonged remission duration after high-dose cytarabine intensification in acute myeloid leukemia varies by cytogenetic subtype. Cancer Res. 1998 ; 58i18j : 4173-9.i2Diij

3j Byrd JC, et al. Patients with t (8;21)(q22;q22) and acute myeloid leukemia have superior failure-free and overall survival when repetitive cycles of high-dose cytarabine are administered. J Clin Oncol. 1999 ; 17i12j : 3767-75.i3iiiDiij

4j Miyawaki S, et al. A randomized comparison of 4 courses of standard-dose multiagent chemotherapy versus 3 courses of high-dose cytarabine alone in postremission therapy for acute myeloid leukemia in adults : the JALSG AML201 Study. Blood. 2011 ; 117i8j : 2366-72.i1iiDiij

5j Schaich M, et al. Cytarabine dose of 36g/m i2j compared with 12 g/m i2j within first consolidation in acute myeloid leukemia : results of patients enrolled onto the prospective randomized AML96 study. J Clin Oncol. 2011 ; 29i19j : 2696-702.i1iiAj


CQ7

Š°‰šŒć—Ć–@‚Ę‚µ‚Ä‚ĢƒVƒ^ƒ‰ƒrƒ“‘å—Ź—Ć–@‚Ģ“Š—^—ŹC•W€“I‰ń”‚Ø‚ę‚ŃŠśŠŌ‚Ķ‰½‚ŖŠ©‚ß‚ē‚ź‚é‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[3
ƒVƒ^ƒ‰ƒrƒ“‘å—Ź—Ć–@‚Ģ•W€“I‰ń”‚ĘŠśŠŌ‚ÉŠÖ‚µ‚Ä–¾Šm‚ČŠī€‚Ķ‘¶Ż‚µ‚Č‚¢BCBF ”’ŒŒ•a‚Å‚ĶƒVƒ^ƒ‰ƒrƒ“‘å—Ź—Ć–@‚Ķ3 ƒR[ƒXˆČć‚Ŗ„§‚³‚ź‚éB

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ƒVƒ^ƒ‰ƒrƒ“iAraCj‘å—Ź—Ć–@‚ĶCAML ‚ĢŠ°‰šŒć—Ć–@‚Ę‚µ‚ĉ¢•Ä‚Å‚Ķ•W€Ž”—Ć‚Ę‚Č‚Į‚Ä‚¢‚éB‚»‚Ģƒ‰ƒ“ƒhƒ}[ƒN‚Ę‚Č‚Į‚½Œ¤‹†‚Ķ1994 ”N‚ÉCancer and Leukemia Study Group BiCALGBj‚©‚ē•ń‚³‚ź‚½Œ¤‹†‚Å‚ ‚é1jB‚±‚ĢŒ¤‹†‚Å‚ĶŠ°‰šĒ—į‚É‘Ī‚µ‚ÄAraC 3 g/m2 6 ‰ń‚š4 ƒR[ƒXŒJ‚č•Ō‚µ‚½•ū‚ŖC400 mg/m2/“ś‚Ü‚½‚Ķ100 mg/m2 Ž‘±“_“H5 “śŠŌ‚š4 ‰ńŒJ‚č•Ō‚µ‚½Ž”—Ć‚ę‚č‚ą—LˆÓ‚É–³•a¶‘¶ŠśŠŌiDFSj‚Ę‘S¶‘¶ŠśŠŌiOSj‚Ŗ—ĒD‚Å‚ ‚Į‚½B‚µ‚©‚µC‚±‚ĢŒ¤‹†‚Å‚Ķ‚Ē‚Ģ‚­‚ē‚¢AraC ‚šŒJ‚č•Ō‚¹‚Ī—Ē‚¢‚Ģ‚©‚ĶŽ¦‚³‚ź‚Ä‚¢‚Č‚¢BCALGB ‚Ķ‚³‚ē‚É9222 Œ¤‹†‚ŁC3 ƒR[ƒX‚ĢAraC ‘å—Ź—Ć –@i3 g/m2 5 “śŠŌ‚Å6 ‰ń“Š—^j‚Ę1 ƒR[ƒX‚ĢAraC ‘å—Ź—Ć–@‚ĢŒćC2 ƒR[ƒX‚Ģ‘½Ü•¹—p‰»Šw—Ć–@‚šs‚¤ŒQ‚Ę‚Å”äŠrŽŽŒ±‚šs‚Į‚½‚ŖC—¼ŒQŠŌ‚ÅDFS/OS ‚Ę‚ą“ŒvŠw“I‚Č—LˆÓ·‚Ķ”F‚ß‚Č‚©‚Į‚½2jB AraC ‘å—Ź—Ć–@‚Ģ•W€“I‚ȉń”‚āŠśŠŌ‚ÉŠÖ‚·‚é‘O•ūŽ‹“IŽŽŒ±‚Ķ‚Č‚­C‚Ü‚½CAraC ‘å—Ź—Ć–@‚ĘƒAƒ“ƒgƒ‰ƒTƒCƒNƒŠƒ“‚šŠÜ‚Ž‘½Ü•¹—p—Ć–@‚Ģ”äŠrŽŽŒ±‚Å‚ą—DˆŹ«‚ĶŽ¦‚³‚ź‚Ä‚¢‚Č‚¢3j`5jBˆź•ūCt (8;21) AML ‚Å‚Ķ3 ƒR[ƒXˆČć‚ĢAraC ‘å—Ź—Ć–@‚Ķ1 ƒR[ƒX‚ĢAraC ‘å—Ź—Ć–@‚ę‚萬Ń‚Ķ—ĒD‚Å‚ ‚é6jB‰¢•Ä‚Å‚ĢAraC ‘å—Ź—Ć–@‚Ķ1 ‰ń“Š—^—Ź‚Ŗ3 g/m2 ‚Å‚ ‚邱‚Ę‚Ŗ‘½‚¢‚ŖC‚±‚Ģ•ū–@‚Å‚Ķ’†•_ŒoŒn‡•¹Ē‚Ŗ‘½‚¢‚Ę‚³‚ź‚Ä‚Ø‚čC‚ķ‚Ŗ‘‚Å‚Ķ•ŪŒÆć”F‚ß‚ē‚ź‚Ä‚¢‚é—p—Ź‚Ķ2 g/m2 ‚Å‚ ‚éB‚±‚ź‚ē‚ĢŽ–ī‚šl—¶‚µ‚ÄŽ”—Ć‚š‘I‘š‚·‚é•K—v‚Ŗ‚ ‚éB

yŽQl•¶Œ£z

1j Mayer RJ, et al. Intensive postremission chemotherapy in adults with acute myeloid leukemia. Cancer and Leukemia Group B. N Engl J Med. 1994 ; 331i14j : 896-903.i1iiAj

2j Moore JO, et al. Sequential multiagent chemotherapy is not superior to high-dose cytarabine alone as postremission intensification therapy for acute myeloid leukemia in adults under 60 years of age : Cancer and Leukemia Group B Study 9222. Blood. 2005 ; 105i9j : 3420-7.i1iiDiij

3j Bradstock KF, et al. A randomized trial of high-versus conventional-dose cytarabine in consolidation chemotherapy for adult de novo acute myeloid leukemia in first remission after induction therapy containing high-dose cytarabine. Blood. 2005 ; 105i2j : 481-8.i1iiDiij

4j Schaich M, et al. Cytarabine dose of 36 g/m i2j compared with 12 g/mi2j within first consolidation in acute myeloid leukemia : results of patients enrolled onto the prospective randomized AML96 study. J Clin Oncol. 2011 ; 29i19j : 2696-702.i1iiAj

5j Miyawaki S, et al. A randomized comparison of 4 courses of standard-dose multiagent chemotherapy versus 3 courses of high-dose cytarabine alone in postremission therapy for acute myeloid leukemia in adults : the JALSG AML201 Study. Blood. 2011 ; 117i8j : 2366-72.i1iiDiij

6j Byrd JC, et al. Patients with t (8;21)(q22;q22) and acute myeloid leukemia have superior failure-free and overall survival when repetitive cycles of high-dose cytarabine are administered. J Clin Oncol. 1999 ; 17i12j : 3767-75.i3iiiDiij


CQ8

ƒVƒ^ƒ‰ƒrƒ“‘å—Ź—Ć–@ˆČŠO‚ĢAML ’nŒÅ‚ß—Ć–@‚Ķ‰½‚ŖŠ©‚ß‚ē‚ź‚é‚©


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ƒJƒeƒSƒŠ[2B
AML ‘ęˆźŠ°‰šŠś‚Ģ’nŒÅ‚ß—Ć–@‚š”ńŒš·‘Ļ«‚ĢƒAƒ“ƒgƒ‰ƒTƒCƒNƒŠƒ“Œn–ņÜ‚š—p‚¢‚ÄŽ{s‚·‚鏼‡‚ĶC4 ‰ń‚ĢŽ”—Ć‚Ŗ„§‚³‚ź‚éB

y‰š ąz

AML ‚Å‚ĶŠ°‰šŒć’nŒÅ‚ß—Ć–@‚šs‚ķ‚Č‚¢‚ĘÄ”­‚Ŗ•KŽŠ‚Å‚ ‚čCŠ°‰š‚šˆŪŽ‚·‚邽‚ß‚ĢŽķX‚ĢŠ°‰šŒć—Ć–@‚ŖŽŽ‚³‚ź‚Ä‚«‚½B‘‚­‚©‚ē‰¢•Ä‚Å‚ĶƒVƒ^ƒ‰ƒrƒ“iAraCj‘å—Ź—Ć–@‚Ŗ’nŒÅ‚ß—Ć–@‚ĢŽå—¬‚Å‚ ‚Į‚½‚ŖC‚ķ‚Ŗ‘‚Å‚Ķ•ŪŒÆć‚Ģ§–ń‚©‚ēAraC ‘å—Ź—Ć–@‚Ŗs‚¦‚Č‚©‚Į‚½ŽžŠś‚Ŗ’·‚­C”ńŒš·‘Ļ«‚ĢƒAƒ“ƒgƒ‰ƒTƒCƒNƒŠƒ“Œn–ņÜ‚ĘAraC •W€—Ź‚š—p‚¢‚½Š°‰šŒć—Ć–@‚Ŗs‚ķ‚ź‚Ä‚«‚½ŒoˆÜ‚Ŗ‚ ‚éB65 ĪˆČ‰ŗ‚ĢAML Š°‰šĒ—į‚É‘Ī‚µ‚Ä‚ĶJapan Adult Leukemia Study GroupiJALSGj‚ŖAML97 ‚É‚Ø‚¢‚ď]—ˆ‚Ģ3 ‰ń‚Ģ’nŒÅ‚ß—Ć–@{6 ‰ń‚ĢˆŪŽ—Ć–@‘Ī4 ‰ń‚Ģ’nŒÅ‚ß—Ć–@‚šs‚¢CˆŪŽ—Ć–@‚Ķs‚ķ‚Č‚¢Ž”—Ć‚Ę‚Ģƒ‰ƒ“ƒ_ƒ€‰»”äŠrŽŽŒ±‚šŽĄŽ{‚µ‚½B‚±‚ĢŒ‹‰Ź‚Å‚ĶC—¼ŽŅ‚É–³•a¶‘¶Š„‡iDFSj‚É‚ą‘S¶‘¶Š„‡iOSj‚É‚ą‘S‚­·‚š”F‚ß‚Č‚©‚Į‚½1jB‚·‚Č‚ķ‚æC4 ‰ń‚Ģ’nŒÅ‚ß—Ć–@‚šs‚¤‚±‚Ę‚ÅˆŪŽ—Ć–@‚šs‚ķ‚Č‚­‚Ä‚ą“Æ“™‚ĢŽ”—ĆŒų‰Ź‚ŖŠś‘Ņ‚Å‚«‚éB‚³‚ē‚ÉJALSG ‚Å‚Ķ4 ‰ń‚Ģ’nŒÅ‚ß—Ć–@‚Ę3 ‰ń‚ĢAraC ‘å—Ź—Ć–@‚Ę‚Ģƒ‰ƒ“ƒ_ƒ€‰»”äŠrŽŽŒ±‚šs‚¢C—¼Ž”—Ć–@‚ÉDFSCOS ‚É—LˆÓ·‚š”F‚ß‚Č‚©‚Į‚½2jB‚Ü‚½CCancer and Leukemia Study Group BiCALGBj‚Ķ3 ƒR[ƒX‚ĢAraC ‘å—Ź—Ć–@i3 g/m2 5 “śŠŌ‚Å6 ‰ń“Š—^j‚Ę1 ƒR[ƒX‚ĢAraC ‘å—Ź—Ć–@{2 ƒR[ƒX‚Ģ‘½Ü•¹—p‰»Šw—Ć–@‚šs‚¤ŒQ‚Ę‚Ģ”äŠrŽŽŒ±‚šs‚Į‚½‚ŖCDFS/OS ‚Ę‚ą‚É‘S‚­·‚š”F‚ß‚Č‚©‚Į‚½3j‚Ę‚µ‚Ä‚¢‚é‚ŖC—¼ŒQ‚Ę‚ą‚ÉAraC ‘å—Ź—Ć–@‚Ŗ“ü‚Į‚Ä‚Ø‚čCƒˆ‚ČŽ”—Ć–@‚Ģ‰ń”‚Ģ”äŠr‚Ķ“ļ‚µ‚¢B

yŽQl•¶Œ£z

1j Miyawaki S, et al. A randomized, postremission comparison of four courses of standard-dose consolidation therapy without maintenance therapy versus three courses of standard-dose consolidation with maintenance therapy in adults with acute myeloid leukemia : the Japan Adult Leukemia Study Group AML 97 Study. Cancer. 2005 ; 104i12j : 2726-34.i1iiDiij

2j Miyawaki S, et al. A randomized comparison of 4 courses of standard-dose multiagent chemotherapy versus 3 courses of high-dose cytarabine alone in postremission therapy for acute myeloid leukemia in adults : the JALSG AML201 Study. Blood. 2011 ; 117i8j : 2366-72.i1iiDiij

3j Moore JO, et al. Sequential multiagent chemotherapy is not superior to high-dose cytarabine alone as postremission intensification therapy for acute myeloid leukemia in adults under 60 years of age : Cancer and Leukemia Group B Study 9222. Blood. 2005 ; 105i9j : 3420-7.i1iiDiij


CQ9

Žį”NŽŅAML ‚Ģ‘ęˆźŠ°‰šŠś‚É“ÆŽķ‘¢ŒŒŠ²×–EˆŚA‚Ģ“K‰ž‚Ķ‚Ē‚Ģ‚ꂤ‚ÉŒˆ’č‚·‚ׂ«‚©


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ƒJƒeƒSƒŠ[1
Œ»Žž“_‚Å‚Ķ‰fŽž‚ĢõF‘ĢˆŁķ‚É‚ę‚銳ŽŅ‘w•Ź‰»‚Ŗd—v‚Å‚ ‚éB—\Œć—ĒD‚ȐõF‘ĢˆŁķ‚š—L‚·‚égood-risk ‚ĢŠ³ŽŅ‚Å‚Ķ‘¢ŒŒŠ²×–EˆŚA‚Ģ—L—p«‚ĶŽ¦‚³‚ź‚Ä‚¢‚Č‚¢B‚»‚źˆČŠO‚ĢAML ‚É‚Ø‚¢‚Ä‚ĶCHLA “K‡ŒŒ‰ŽŅŠŌ“ÆŽķ‘¢ŒŒŠ²×–EˆŚA‚ŖŠ©‚ß‚ē‚ź‚éB

y‰š ąz

‚±‚ź‚܂ŁC‘ęˆźŠ°‰šŠś‚É‚Ø‚Æ‚é“ÆŽķ‘¢ŒŒŠ²×–EˆŚA‚ĘŠ°‰šŒć‰»Šw—Ć–@‚š”äŠr‚µ‚½‘½”‚Ģƒ‰ƒ“ƒ_ƒ€‰»”äŠrŽŽŒ±‚ŖŽ{s‚³‚ź‚½‚ŖCŒ‹‰Ź‚Ķˆź’č‚Å‚Č‚­–³•a¶‘¶Š„‡iDFSj‚ňŚAŒQ‚Ģ—LŒų«‚šŽ¦‚·Œ¤‹†‚Ķ‚ ‚é‚ą‚Ģ‚ĢC‚Ł‚Ę‚ń‚Ē‚ĢŒ¤‹†‚Ķ‘S¶‘¶Š„‡iOSj‚Å‚Ģ—LŒų«‚šŽ¦‚·‚±‚Ę‚Ŗ‚Å‚«‚Č‚©‚Į‚½B‚Ü‚½C‰»Šw—Ć–@‚Ģ—\Œć•s—ĒˆöŽq‚©‚ē—¼ŽŅ‚š”äŠr‚·‚錟“¢‚ą\•Ŗ‚Č“ŒvŠw“IpoweriĒ—į”j‚Ŗ‚Č‚­Œ‹˜_‚š“±‚«o‚·‚±‚Ę‚Ķ‚Å‚«‚Č‚©‚Į‚½B‚µ‚©‚µC24 ‚Ģ—Տ°Œ¤‹†iĒ—į”3,638j‚š‘ĪŪ‚Ę‚µ‚½ƒƒ^ƒAƒiƒŠƒVƒX‚ĢŒ‹‰Ź‚Å‚ĶCAML ‘ęˆźŠ°‰šŠś‚Å‚ĶC—\Œć•s—Ē‚Ø‚ę‚Ń’†ŠŌõF‘ĢˆŁķ‚Ģ‚ ‚éĒ—į‚Å‚ĶCˆŚA‚É‚ę‚鐶‘¶Š„‡‚Ŗ—LˆÓ‚ɏŸ‚é‚ŖC—\Œć—ĒDõF‘ĢˆŁķ‚Ģ‚ ‚éĒ—į‚Å‚ĶˆŚA‚Ģ—DˆŹ«‚ĶŠm”F‚Å‚«‚Č‚©‚Į‚½1jB‚±‚ĢŒ‹‰Ź‚©‚ē‚ĶCõF‘ĢˆŁķ‚Ģ—L–³‚ĘŽķ—Ž‚É‚ę‚Į‚Ä‘ęˆźŠ°‰šŠś‚É‚ĶˆŚA“K‰ž‚šŒˆ’č‚·‚邱‚Ę‚Ŗ•W€“I‚ĘŒ¾‚¦‚éB–Æ‘°ˆā“`Šw“I”wŒi‚ĢˆŁ‚Č‚é‚ķ‚Ŗ‘‚ɉ¢•Ä‚Ģƒf[ƒ^‚šŠO‘}‚·‚邱‚Ę‚Ģ‘Ć“–«‚Ķ\•Ŗ‚ÉŒŸŲ‚³‚ź‚Ä‚¢‚Č‚¢‚ŖC‚ķ‚Ŗ‘‚ÅŽ{s‚³‚ź‚½ƒ‰ƒ“ƒ_ƒ€‰»”äŠrŽŽŒ±‚Å‚ą“Æ—l‚ČŒ‹‰Ź‚Ŗ“¾‚ē‚ź‚Ä‚¢‚éB‚µ‚©‚µC—\ŒćˆöŽq‚Ģ’č‹`‚ŖˆŁ‚Č‚éiõF‘ĢˆŁķ‚Ģ‚Ż‚Å‚Ķ‚Č‚¢j‚±‚ʂɉĮ‚¦‚āCĒ—į‚Ŗ­‚Č‚­ƒRƒ“ƒvƒ‰ƒCƒAƒ“ƒX‚ą’į‚­C—Տ°ŽŽŒ±‚Ę‚µ‚Ä‚ĢŽæ‚Ķ‚‚­‚Č‚¢2jBÅ‹ß‚Å‚ĶCˆā“`Žq•ĻˆŁ‚Ģ—L–³‚ą—\ŒćˆöŽq‚Ę‚µ‚Ä’–Ś‚³‚ź‚Ä‚¢‚éBˆź‚Ā‚Ģƒ‰ƒ“ƒ_ƒ€‰»”äŠrŽŽŒ±‚ĢƒTƒuƒOƒ‹[ƒv‰šĶ‚Å‚ĶC³ķŠjŒ^AML ‘ęˆźŠ°‰šŠś‚É‚Ø‚¢‚ÄNPM1 ‚Ģˆā“`Žq•ĻˆŁ‚Ŗ‚ ‚čFLT3-ITD ‚Ģ‚Č‚¢Ē—į‚šœ‚¢‚½Ē—įŒQ‚Å‚ĶCHLA “K‡ŒŒ‰ŽŅŠŌˆŚAŒć‚Ģ–³Ä”­¶‘¶Š„‡iRFSj‚Ŗ—LˆÓ‚ɏŸ‚邱‚Ę‚ŖŠm”F‚³‚ź‚Ä‚¢‚é3jBCEBPA ‚Ģˆā“`Žq•ĻˆŁ‚ÉŠÖ‚µ‚Ä‚Ģƒf[ƒ^‚Ķ‚Č‚¢B‚Ü‚½C—\Œć—ĒDõF‘ĢŒQ‚É‚Ø‚Æ‚éKIT ‚Ģˆā“`ŽqˆŁķ‚ÉŠÖ‚µ‚Ä‚ąƒf[ƒ^‚Ķ‚Č‚¢B—Տ°‚Å‚Ķ”ńŒŒ‰ŽŅŠŌˆŚA‚ąõF‘ĢˆŁķ‚šŽw•W‚Ę‚µ‚Ä‘I‘š‚³‚ź‚Ä‚¢‚é‚Ģ‚ŖŒ»ó‚Å‚ ‚é‚ŖCŒŒ‰ŽŅŠŌˆŚA‚Ę“Æ“™‚ĢƒGƒrƒfƒ“ƒX‚ĶŠm—§‚³‚ź‚Ä‚¢‚Č‚¢B

yŽQl•¶Œ£z

1j Koreth J, et al. Allogeneic stem cell transplantation for acute myeloid leukemia in first complete remission : a systematic review and meta-analysis of prospective clinical trial. JAMA. 2009 ; 30 i22j : 2349-61.i1iiAj

2j Sakamaki H, et al. Allogeneic stem cell transplantation versus chemotherapy as post-remission therapy for intermediate or poor risk adult acute myeloid leukemia : results of the JALSG AML97 study. Int J Hematol. 2010 ; 91i2j : 284-92.i1iiDij

3j Schlenk RF, et al. Mutations and treatment outcome in cytogenetically normal acute myeloid leukemia. N Engl J Med. 2008 ; 358i18j : 1909-18.i2Diiij


CQ10

ˆŚA“K‰ž‚Ģ‚Č‚¢‚—īŽŅAML ‚ÉŠ°‰šŒć—Ć–@‚šŽ{s‚·‚郁ƒŠƒbƒg‚Ķ‚ ‚é‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2B
ˆŚA“K‰ž‚Ģ‚Č‚¢‚—īŽŅAML ‚É‘Ī‚·‚銰‰šŒć—Ć–@‚ĢƒƒŠƒbƒg‚Ķ–¾‚ē‚©‚É‚³‚ź‚Ä‚¢‚Č‚¢‚ŖCˆź•”‚ĢĒ—į‚Å‚ĶŠ°‰šŒć—Ć–@‚šs‚¤‚±‚Ę‚Ģ—L—p«‚ŖŽ¦“‚³‚ź‚Ä‚¢‚éB

y‰š ąz

Cancer and Leukemia Study Group BiCALGBj‚ĶŠ°‰šŒć—Ć–@‚Ę‚µ‚ăVƒ^ƒ‰ƒrƒ“‘å—Ź“Š—^iAraC 3 g/m2 6 ‰ń‚š4 ƒR[ƒXŒJ‚č•Ō‚·j‚Ģ—L—p«‚šŒŸ“¢‚µC60 ĪˆČć‚ĢĒ—į‚É‚Ø‚Æ‚éAraC ‘å—Ź“Š—^‚Ķ‚»‚Ģ•W€—Ź“Š—^‚Ę”äŠr‚µ‚Ä–³•a¶‘¶—¦iDFSj‚š‰ü‘P‚µ‚Č‚¢‚±‚Ę‚š–¾‚ē‚©‚É‚µ‚½1jB‚—īŽŅAML ‚š‘ĪŪ‚Ę‚µ‚½MRC-AML 11 ŽŽŒ±‚ą1 ‰ń‚Ģ‹­‰»—Ć–@Œć‚É“Æ—l‚Ģ‰»Šw—Ć–@‚šŒJ‚č•Ō‚µ‚Ä‚ąÄ”­—¦CDFSCOS ‚É—LˆÓ·‚Ķ‚Ż‚ē‚ź‚Č‚¢‚±‚Ę‚šŽ¦‚µ‚Ä‚¢‚é2jBˆź•ūCAML HD98-B ŽŽŒ±‚ĶŠ°‰š’B¬Œć‚Ģ‹­‰»—Ć–@‚É‚ę‚Į‚āC—\Œć—ĒDõF‘ĢˆŁķ‚šŽ‚Ā‚—īŽŅAML ‚Ģ20`30“‚É’·Šś¶‘¶‚Ŗ“¾‚ē‚ź‚邱‚Ę‚šŽ¦‚µ‚Ä‚Ø‚č3jC“Æ—l‚ĢŒXŒü‚ĶMRC-AML11 ŽŽŒ±‚É‚Ø‚¢‚Ä‚ąŠm”F‚³‚ź‚Ä‚¢‚éB‚±‚ź‚ē‚ĢŒ¤‹†‚©‚ē‚ĶCˆź•”‚Ģ‚—īŽŅAML ‚Å‚ĶŠ°‰šŒć—Ć–@‚šŽ{s‚·‚郁ƒŠƒbƒg‚Ŗ‚ ‚邱‚Ę‚ŖŽ¦“‚³‚ź‚éB‚µ‚©‚µCõF‘ĢˆŁķˆČŠO‚ĢŠ³ŽŅ‘I‘š‚ÉŠÖ‚·‚éŽw•Wi”N—īC•¹‘¶Ē‚Č‚Ēj‚Ķ–¾Šm‚É‚³‚ź‚Ä‚¢‚Č‚¢BŽŠ“K‚ČŠ°‰šŒć—Ć–@‚ÉŠÖ‚µ‚Ä‚ąŠm—§‚µ‚½ƒGƒrƒfƒ“ƒX‚Ķ‚Č‚¢‚ŖCAcute Leukemia French AssociationiALFAj9803 ŽŽŒ±‚Ķ‹­—Ķ‚ȉ»Šw—Ć–@1 ‰ń‚ĘŠO—ˆ‚Å‚Ģ‰»Šw—Ć–@6 ‰ń‚š”äŠr‚µCŒćŽŅ‚ÅŠ°‰šŒć2 ”N‚ĢOS ‚Ŗ—D‚źCÄ”­‚É‚Ķ·‚Ŗ‚Č‚¢‚ą‚Ģ‚ĢCŽ”—ĆŠÖ˜AŽ€–SiTRMj‚ĶŠO—ˆ‰»Šw—Ć–@‚ŏ­‚Č‚©‚Į‚½‚Ę•ń‚µ‚Ä‚¢‚é4jB

yŽQl•¶Œ£z

1j Mayer RJ, et al. Intensive postremission chemotherapy in adults with acute myeloid leukemia. Cancer and Leukemia Group B. N Engl J Med. 1994 ; 331i14j : 896-903.i1iiAj

2j Goldstone AH, et al. Attempts to improve treatment outcomes in acute myeloid leukemiaiAMLj in older patients : the results of the United Kingdom Medical Research Council AML 11 trial. Blood. 2001 ; 98i5j : 1302-11.i1iiAj

3j Frohling S, et al. Cytogenetics and age are major determinants of outcome in intensively treated acute myeloid leukemia patients older than 60 years : results from AMLSG trial AMD HD98-B. Blood. 2006 ; 108i10j : 3280-8.i2Aj

4j Gardin C, et al. Postremission treatment of elderly patients with acute myeloid leukemia in first complete remission after intensive induction chemotherapy : results of the multicenter randomized Acute Leukemia French AssociationiALFAj 9803 trial. Blood. 2007 ; 109i12j : 5129-35.i1iiAj


CQ11

”ńŠ°‰šŠśAML ‚É‘Ī‚·‚é“ÆŽķ‘¢ŒŒŠ²×–EˆŚA‚Ģ“K‰ž‚ÉŠÖ‚·‚éŽw•W‚Ķ‚ ‚é‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[3
”ńŠ°‰šŠśAML ‚É‘Ī‚·‚éˆŚA“K‰ž‚šŒˆ’č‚·‚é–¾Šm‚ČŽw•W‚ĶŠm—§‚³‚ź‚Ä‚¢‚Č‚¢BŒ»Žž“_‚Å‚ĶŒć•ūŽ‹“I‰šĶ‚ÉŠī‚Ć‚­—\ŒćˆöŽq‚ĘˆŚA‚ÉŠÖ˜A‚·‚éˆöŽqiƒhƒi[ƒ\[ƒX‚Č‚Ēj‚š‘‡“I‚É•]‰æ‚µCŠ³ŽŅ‚Ę‚Ģshared decision making ‚ňŚA“K‰ž‚šŒˆ‚߂邱‚Ę‚ŖŠ©‚ß‚ē‚ź‚éB”ńŠ°‰šŠśAML ‚É‚Ø‚¢‚ĈŚA‘O‚ɉ»Šw—Ć–@‚šŽ{s‚·‚邱‚Ę‚ĢƒƒŠƒbƒg‚Ŗ‚ ‚éĒ—į‚š—\‘Ŗ‚·‚éŽw•W‚ĶŠm—§‚µ‚Ä‚¢‚Č‚¢B

y‰š ąz

‰‰ńÄ”­Šś‚É‚Ø‚¢‚Ä“ÆŽķ‘¢ŒŒŠ²×–EˆŚA‚ʉ»Šw—Ć–@‚Ģ—L—p«‚š‘O•ūŽ‹“I‚É”äŠrŒŸ“¢‚µ‚½•ń‚Ķ‚Č‚¢BBreems ‚ē‚Ķ15`60 Ī‚Ģ‰‰ńÄ”­AML ‚ĢˆŚA¬Ń‚šŒć•ūŽ‹“I‚ɉšĶ‚µC4 ‚Ā‚Ģ—\ŒćˆöŽqi‘ęˆźŠ°‰š‚ĢŠśŠŌCf’fŽž‚ĢõF‘ĢˆŁķC‰‰ńÄ”­Žž‚Ģ”N—īC‰‰ńÄ”­‘O‚Ģ‘¢ŒŒŠ²×–EˆŚA‚Ģ—L–³j‚š“Æ’č‚µC‚±‚ź‚šŠī‚ɏ‰‰ńÄ”­Šś‚š3 ‚Ā‚Ģ—\ŒćƒOƒ‹[ƒv‚É•Ŗ—Ž‚µ‚Ä‚¢‚éifavorableCintermediateCunfavorablejB‘ę“ńŠ°‰šŠś‚Ŗ’B¬‚Å‚«‚½Ē—į‚ÉŒĄ’肵‚ĈŚA‚ʉ»Šw—Ć–@‚š”äŠr‚µ‚½ŒŸ“¢‚Å‚ĶC‚¢‚ø‚ź‚ĢŒQ‚É‚Ø‚¢‚Ä‚ą5 ”N‘S¶‘¶Š„‡iOSj‚ňŚA‚Ģ—DˆŹ«‚ŖŽ¦‚³‚ź‚Ä‚¢‚é1jB“ś–{‚Ģ‰‰ńÄ”­ŠśAML ‚ĢˆŚA¬Ń‚ĢŒć•ūŽ‹“I‰šĶ‚ŁC‘ę“ńŠ°‰šŠś‚š’B¬‚·‚邱‚Ę‚Å3 ”NOS ‚Ķ—LˆÓ‚ɉü‘P‚·‚邱‚Ę‚Ŗ•ń‚³‚ź‚Ä‚¢‚é2jBˆź•ūC‰č‹…‚ĢŠ„‡‚Ģ­‚Č‚¢‰‰ńÄ”­Šś‚É“ÆŽķˆŚA‚šs‚¤‚±‚Ę‚Å‘ę“ńŠ°‰šŠś‚Ę“Æ“™‚Ģ¶‘¶Š„‡‚Ŗ“¾‚ē‚ź‚é‚Ę‚Ģ•ń‚ą‚ ‚é‚ŖC‚»‚ĢƒGƒrƒfƒ“ƒXƒŒƒxƒ‹‚Ķ’į‚¢3jB”ńŠ°‰šŠśAML ‚É‘Ī‚·‚é“ÆŽķ‘¢ŒŒŠ²×–EˆŚA‚Ģ¬Ń‚ĶŽUŒ©‚³‚ź‚é‚ŖC‚·‚ׂĂŖ­”—į‚Å‚Ģ•ń‚Ŗ‘½‚¢‚±‚Ę‚ĘŠ³ŽŅ‚Ģselection bias ‚É‚ę‚čC‚»‚ĢˆŚA“K‰ž‚ĢŽw•W‚š“±‚«o‚·‚±‚Ę‚Ķ“ļ‚µ‚¢B‚µ‚©‚µthe Center for International Blood and Marrow Transplant ResearchiCIBMTRj‚ĶAML ”ńŠ°‰šŠśˆŚA1,673 —į‚š‰šĶ‚µC‘ęˆźŠ°‰šŠś‚ĢŠśŠŌC––½ŒŒ’†‚Ģ‰č‹…Š„‡Cƒhƒi[‚ĢŽķ—ŽCPSCõF‘ĢˆŁķ‚Ģ—L–³‚©‚ēCˆŚAŒć‚Ģ3 ”NOS 42“‚Ģ—\Œć—ĒDŒQ‚©‚ē6“‚Ģ—\Œć•s—ĒŒQ‚Ü‚Å‚ĢŠ³ŽŅ‚Ģ‘w•Ź‰»‚Ŗ‚ ‚é’ö“x‰Ā”\‚Å‚ ‚邱‚Ę‚š•ń‚µ‚Ä‚¢‚é4jB

yŽQl•¶Œ£z

1j Breems DA, et al. Prognostic index for adults patients with acute myeloid leukemia in first relapse. J Clin Oncol. 2005 ; 23i9j : 1969-78.i2Aj

2j Kurosawa S, et al. Prognostic factors and outcomes of adults patients with acute myeloid leukemia after first relapse. Hematologica. 2010 ; 95i11j : 1857-64.i3iAj

3j Clift RA, et al. Allogeneic marrow transplantation during untreated first relapse of acute myeloid leukemia. J Clin Oncol. 1992 ; 10i11j : 1723-9.i3iiAj

4j Duval M, et al. Hematopoietic stem-cell transplantation for acute leukemia in relapse or primary induction failure. J Clin Oncol. 2010 ; 28i23j : 3730-8.i3iAj


CQ12

AML ‚É‚Ø‚¢‚ÄŽ”—ĆŒć‚ĢD’†‹…Œø­Šś‚ÉG-CSF ‚šŽg—p‚·‚é‚Ģ‚Ķ—L—p‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2B
iŠ°‰š“±“ü—Ć–@j
ƒJƒeƒSƒŠ[2A
iŠ°‰šŒć—Ć–@j
AML ‚ĢŠ°‰š“±“ü—Ć–@CŠ°‰šŒć—Ć–@Žž‚É‚Ø‚Æ‚éG-CSF “Š—^‚ĶCD’†‹…Œø­ŠśŠŌ‚Ģ’Zk‚āQOL ‚Ģ‰ü‘P‚ŖŠś‘Ņ‚Å‚«C‚—īŽŅ‚ādĒŠ“õĒ‚š•¹”­‚µ‚½Ē—į‚Å‚ĶŒŸ“¢‚µ‚Ä‚ą—Ē‚¢B

y‰š ąz

AML Ž”—Ć‚ĶCŠ°‰š“±“ü—Ć–@CŠ°‰šŒć—Ć–@‚Ę‚ą‚É‹­‰»‚³‚źCŠ°‰š—¦‚Ķ80“C5 ”N‘S¶‘¶Š„‡iOSj‚Ķ50“‘OŒć‚ĘŒüć‚µ‚Ä‚¢‚éB‚±‚ĢŽ”—Ć‚Ģ‹­‰»‚ĶCœ‘—}§‚É‚ę‚éoŒŒ‚āˆÕŠ“õ«‚Ģ‘Īō‚ĢŒüć‚É‚ę‚č‰Ā”\‚É‚Č‚Į‚½B

‚±‚ĢAML ‚ĢŠ°‰š“±“ü—Ć–@‚ā’nŒÅ‚ß—Ć–@Œć‚ɏoŒ»‚·‚銓õĒ‚š—\–h‚Å‚«‚é‚©‚Ē‚¤‚©C‚±‚ź‚Ü‚Å‚É‚¢‚­‚Ā‚©‚ĢG-CSF “Š—^‚Ģ‘ęⅢ‘ŠŽŽŒ±‚ŖŽĄŽ{‚³‚ź‚½B

Žį”N¬lAML ‚š‘ĪŪ‚Ę‚µ‚½‘ęⅢ‘ŠŽŽŒ±‚Å‚ĶCD’†‹…”Œø­ŠśŠŌC”­”MŠśŠŌC”ńŒoŒūR¶Ü‚Ģ“Š—^ŠśŠŌ‚³‚ē‚É‚Ķ“ü‰@ŠśŠŌ‚Ģ’Zk‚ŖŽ¦‚³‚ź‚Ä‚¢‚é1jB‚ķ‚Ŗ‘‚ōs‚ķ‚ź‚½Œ¤‹†‚Å‚ąCD’†‹…”Œø­ŠśŠŌC”­”MŠśŠŌ‚Ģ’Zk‚ŖŠĻŽ@‚³‚ź‚Ä‚¢‚é2jB

œ‘—}§‚Ŗ‚“x‚Ę‚Č‚é‚—īŽŅAML ‚š‘ĪŪ‚É‚µ‚½ŽŽŒ±‚Å‚ąCD’†‹…”Œø­ŠśŠŌC”­”MŠśŠŌC”ńŒoŒūR¶Ü‚Ģ“Š—^ŠśŠŌ‚Ģ’Zk‚Ŗ”F‚ß‚ē‚ź‚Ä‚¢‚é3j4jB‚Ü‚½CŽ€–S—¦‚ĶŒø­‚µ‚Č‚©‚Į‚½‚ŖCŠ°‰š—¦‚ĶŒüć‚µ‚½‚Ę‚Ģ•ń‚ą‚ ‚é5jB

AML ×–E‚ĶCG-CSF Žó—e‘Ģ‚š”­Œ»‚·‚邱‚Ę‚©‚ēCAML ‚Ö‚ĢG-CSF ‚Ģ“Š—^‚Ķ–ā‘莋‚³‚ź‚Ä‚¢‚é‚ŖCÄ”­—¦‚Ģ‘‰Į‚Ķ‚Ż‚ē‚ź‚øC’·ŠśŠĻŽ@‚É‚Ø‚¢‚Ä‚ą¶‘¶ŠśŠŌ‚Ɉ«‰e‹æ‚š—^‚¦‚Ä‚¢‚Č‚¢‚Ę•ń‚³‚ź‚Ä‚¢‚é6jB

AML ‚ĢŠ°‰š“±“üC’nŒÅ‚ß—Ć–@Žž‚É‚Ø‚¢‚Ä‚ĶCG-CSF “Š—^‚É‚ę‚čD’†‹…Œø­ŠśŠŌ‚Ķ’Zk‚·‚é‚ą‚Ģ‚ĢCdĒŠ“õĒ‚Ģ”­Ē—¦‚āŽ€–S—¦‚ĶŒø­‚¹‚øC¶‘¶ŠśŠŌ‚Ģ‰„’·‚ą”F‚ß‚ē‚ź‚Ä‚¢‚Č‚¢B‚µ‚½‚Ŗ‚Į‚āCEuropean LeukemiaNet ‚ĢŠ©‚āNational Comprehensive Cancer NetworkiNCCNj‚ĢƒKƒCƒhƒ‰ƒCƒ“‚Å‚Ķ‚—īŽŅ‚ādĒŠ“õĒ‚š•¹”­‚µ‚½Ē—įˆČŠO‚ĢAML Ē—į‚Ö‚ĢG-CSF ‚Ģ“Š—^‚Ķ„§‚µ‚Ä‚¢‚Č‚¢B‚µ‚©‚µCAmerican Society of Clinical OncologyiASCOj‚ĢƒKƒCƒhƒ‰ƒCƒ“‚Å‚ĶŠ°‰š“±“ü—Ć–@Œć‚ĢG-CSF “Š—^‚Ķ‘Ć“–C’nŒÅ‚ß—Ć–@Œć‚Ķ„§‚Å‚«‚é‚Ę‚µ‚Ä‚¢‚é7jB

ˆź•ūCŽ”—ĆŒć‚ĢD’†‹…Œø­Šś‚Éč÷—±‹…‚š—A’‚·‚邱‚Ę‚É‚ę‚銓õĒ”­Ē—\–h‚ĢŽŽŒ±‚ŖŽĄŽ{‚³‚źC­”—į‚ĢŒŸ“¢‚Å‚Ķ‚»‚Ģ—LŒų«‚ŖŽ¦‚³‚ź‚½8jB‚µ‚©‚µC‘½”—į‚ĢŒŸ“¢‚Å‚Ķ×‹Ū«‚Ģ”sŒŒĒ‚ĢŒø­‚Ģ‚Ż‚ŁC‚»‚Ģ‘¼‚ĢŠ“õĒ‚Ģ”­Ē‚ĶŒø­‚¹‚øC—LŠQŽ–Ū‚Ģ•p“x‚Ŗ‚‚©‚Į‚½‚Ę‚Ģ•ń9j‚Ŗ‚ ‚čC‚»‚Ģ•]‰æ‚Ķˆź’肵‚Č‚¢B

yŽQl•¶Œ£z

1j Heil G, et al. A randomized, double-blind, placebo-controlled, phase Ⅲ study of filgrastim in remission induction and consolidation therapy for adults with de novo acute myeloid leukemia. The International Acute Myeloid Leukemia Study Group. Blood. 1997 ; 90i12j : 4710-8.i1iDj

2j Usuki K, et al. Efficacy of granulocyte colony-stimulating factor in the treatment of acute myelogenous leukemia : multicentre randomized study. Br J Haematol. 2002 ; 116i1j : 103-12.i1iiDj

3j Growin JE, et al. A double-blind placebo-controlled trial of granulocyte colony-stimulating factor in elderly patients with previously untreated acute myeloid leukemia. : A Southwest Oncology Group Study i9031j. Blood. 1998 ; 91i10j : 3607-15.i1iDj

4j Amadori S, et al. Use of glycosylated recombinant human G-CSF ilenograstimj during and/or after induction chemotherapy in patients 61 years of age and older with acute myeloid leukemia : final results of AML-13. A randomized phase-3 study. Blood. 2005 ; 106i1j : 27-34.i1iiDj

5j Dombret H, et al A controlled study of recombinant human granulocyte colony-stimulating factor in elderly patients after treatment for acute myelogenous leukemia. N Eng J Med. 1995 ; 332 i25j : 1678-83.i1iiDj

6j Heil G, et al. Long-term survival data from a phase 3 study of Filgrastim as an adjunct to chemotherapy in adults with de novo acute myeloid leukemia. Leukemia. 2006 ; 20i3j : 404-9.i1iDj

7j Smith TJ, et al. 2006 update of recommendations for the use of white blood cell growth factor : an evidence-based clinical practice guide line. J Clin Oncol. 2006 ; 24i19j : 3187-205.iƒKƒCƒhƒ‰ƒCƒ“j

8j Gomez-Villagran JL, et al. A controlled trial of prophylactic granulocyte transfusions during induction chemotherapy for acute nonlymphoblastic leukemia. Cancer. 1984 ; 54i4j : 734-8.i3iDj

9j Strauss RG, et al. A controlled trial of prophylactic granulocyte transfusions during initial induction chemotherapy for acute myelogenous leukemia. N Eng J Med. 1981 ; 305i11j : 597-603.i1iiDj


CQ13

AML ‚Ģ‰»Šw—Ć–@‚É‚Ø‚¢‚āC‚Ē‚Ģ‚ꂤ‚ȏź‡‚ÉŽīį‡•ö‰óĒŒóŒQ‚Ģ—\–h‚šŽĄŽ{‚·‚ׂ«‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2A
AML ‚Ģ‰»Šw—Ć–@ŽžC––½”’ŒŒ‹…”‚Ŗ‚’l–‚ ‚é‚¢‚Ķ‹}‘‚µC”AŽ_’l‚āŒŒ“ƒNƒŒƒAƒ`ƒjƒ“’l‚Ŗćø‚µ‚Ä‚¢‚éĒ—į‚É‘Ī‚µ‚Ä‚ĶŽīį‡•ö‰óĒŒóŒQ‚Ģ—\–h‚Ŗ„§‚³‚ź‚éB

y‰š ąz

Žīį‡•ö‰óĒŒóŒQ‚ĶŽīį‡×–E‚Ģ”j‰ó‚É‚ę‚č”AŽ_CƒŠƒ“Ž_CƒJƒŠƒEƒ€‚Č‚Ē‚ŖŒŒ‰t’†‚Ɉź‹C‚É•śo‚³‚źC‚”AŽ_ŒŒĒC‚ƒŠƒ“ŒŒĒC‚ƒJƒŠƒEƒ€ŒŒĒó‘Ō‚Ę‚Č‚čC‹}«t•s‘S‚āŒÄ‹z•s‘SCź‡‚É‚ę‚Į‚Ä‚ĶS’āŽ~‚Ŗˆų‚«‹N‚±‚³‚ź‚éĒŒóŒQ‚Å‚ ‚éB‚µ‚½‚Ŗ‚Į‚āC‘å—Ź‚ĢŽīį‡×–E‚Ŗ’ZŽžŠŌ‚Å”j‰ó‚³‚ź‚鏼‡‚ɁCŽīį‡•ö‰óĒŒóŒQ”­Ē‚ĢƒŠƒXƒN‚Ŗ‚‚­‚Č‚éBŒŒ‰tˆ««Žīį‡‚Å‚ĶC‘BƒXƒs[ƒh‚Ŗ‘‚­CRŽīį‡–ņ‚ÉŠ“Žó«‚Ģ‚‚¢ƒŠƒ“ƒpŒnŽīį‡‚ĢŽ”—Ć‚ÉŪ‚µ‚Ä”­Ē‚·‚éŠm—¦‚Ŗ‚‚¢B

”AŽ_’l‚āŒŒ“ƒNƒŒƒAƒ`ƒjƒ“’l‚Ŗćø‚µ‚Ä‚¢‚éĒ—į‚ĢŠ°‰š“±“ü—Ć–@‚ĢŽžŠś‚É‚Ķ“Į‚É’ˆÓ‚Ŗ•K—v‚Å‚ ‚é1j2jB

Žīį‡•ö‰óĒŒóŒQ‚š—\–h‚·‚邽‚ß‚É‚ĶC‘‰Į‚µ‚½”’ŒŒ•a×–E‚šŠÉ‚ā‚©‚ÉŒø­‚³‚¹‚邱‚Ę‚Ŗ—LŒų‚ŁC“ą•ž–ņ‚ĢƒqƒhƒƒLƒVƒEƒŒƒA‚Å‚ĢŽ”—Ć‚ŖŠ©‚ß‚ē‚ź‚Ä‚¢‚éB‚Ü‚½C…•Ŗ•ā‹‹‚ā”AŽ_‚Ģ¶¬‚šŒø‚ē‚·‚ׂ­ƒAƒƒvƒŠƒm[ƒ‹‚ą“Š—^‚³‚ź‚éB‹@ŠB“I‚É”’ŒŒ•a×–E‚šœ‹Ž‚·‚éƒAƒtƒFƒŒ[ƒVƒX‚ąŠ©‚ß‚ē‚ź‚Ä‚¢‚é‚ŖC‚»‚ĢŒų‰Ź‚ĶˆźŽž“I‚ŁC‘ŠśŽ€–S‚ĶŒø­‚³‚¹‚é‚Ŗ’·Šś—\Œć‚Ķ‰ü‘P‚µ‚Č‚¢‚Ę•ń‚³‚ź‚Ä‚¢‚é3j4jB‚Ü‚½CŽīį‡•ö‰óĒŒóŒQ‚ĢƒnƒCƒŠƒXƒNĒ—į‚É‚Ķ”AŽ_‚š•Ŗ‰š‚µ…—n«‚ĢƒAƒ‰ƒ“ƒgƒCƒ“‚É‚·‚郉ƒXƒuƒŠƒJ[ƒ[‚ĢŽg—p5j‚ŖNCCN ƒKƒCƒhƒ‰ƒCƒ“‚āEuropean LeukemiaNet ‚ĢŠ©C“ś–{—Տ°Žīį‡Šw‰ļ‚ĢŽīį‡•ö‰óĒŒóŒQiTLSjf—ĆƒKƒCƒ_ƒ“ƒX‚É‚ą‹LŚ‚³‚ź‚Ä‚¢‚éB

–”’ŒŒ‹…’˜‘ihyperleukeocytosisj‚ĶCˆź”Ź‚Ɂ„10 –œ/ƒŹL ‚Ę’č‹`‚³‚ź‚éB

yŽQl•¶Œ£z

1j Seftel MD, et al. Fulminant tumor lysis syndrome in acute myelogenous leukaemia with inv (16jip13;q22j. Eur J Haematol. 2002 ; 69i4j : 193-9.i3iDj

2j Montesinos P, et al. Tumor lysis syndrome in patients with acute myeloid leukemia : identifi cation of risk factors and development of a predictive model. Haematologica. 2008 ; 93i1j : 67-74.i3iDj

3j Bug G, et al. Impact of leukapheresis on early death rate in adult acute myeloid leukemia presenting with hyperleukocytosis. Transfusion. 2007 ; 47i10j : 1843-50.i3iiiDj

4j Giles FJ, et al. Leukapheresis reduces early mortality in patients with acute myeloid leukemia with high white cell counts but does not improve long-term survival. Leuk Lymphoma. 2001 ; 42i1-2j : 67-73.i3iiiBj

5j Wang LY, et al. Recombinant urate oxidase iRasburicasej for the prevention and patients with hematologic malignacies. Acta Haematol. 2006 ; 115i1-2j : 35-8.i2Dj


CQ14

AML ‚É‚Ø‚¢‚Ä’†•_ŒoŒn”’ŒŒ•a‚Ģ—\–h‚ĶŠ©‚ß‚ē‚ź‚é‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2A
AML ‚Ķ’†•_ŒoŒn”’ŒŒ•a‚Ģ”­Ē•p“x‚Ŗ’į‚­C‚·‚ׂĂĢĒ—į‚É‘Ī‚µ‚čR‚Ŗ‚ńÜ‚Ģ‘o“ą“Š—^‚É‚ę‚é—\–h‚Ķ„§‚³‚ź‚Ä‚¢‚Č‚¢‚ŖCFAB •Ŗ—Ž‚Å‚ĢM4CM5Cbiphenotype ‚ā––½ŒŒ’†”’ŒŒ‹…‚Ŗ10 –œ/ƒŹL ‚š’“‚¦‚éĒ—į‚Å‚Ķ—\–hŽ”—Ć‚Ŗ„§‚³‚ź‚éB

y‰š ąz

’†•_ŒoŒn”’ŒŒ•a‚ĶC”’ŒŒ•a×–E‚Ŗ”]“ī–Œ‚ɐZ‚µC’Źķ‘–Œ”’ŒŒ•a‚Ę‚µ‚Ä”­Ē‚·‚éB‚µ‚©‚µC‹H‚É‚Ķ”]CŅ‘ŽĄŽæ‚ɐZ‚µŽīįŽ‚šŒ`¬‚·‚邱‚Ę‚ą‚ ‚éBf’f‚ĶC”]Ņ‘‰t’†‚É”’ŒŒ•a×–E‚šŠm”F‚·‚邱‚Ę‚Å‚Č‚³‚źC”]Ņ‘ˆ³‚Ģćø‚É‹Nˆö‚·‚é“Ŗ’ɁCšq“fC“ū“Ŗ•‚ŽīC€•”d’¼‚Č‚Ē‘–ŒŽhŒƒĒóC”]_Œo–ƒįƒ‚Č‚Ē‚ŖŽå‚ȏĒó‚Å‚ ‚éB”­ĒŽžŠś‚Ķ”’ŒŒ•a‚Ģ”­Ē‚Ę“ÆŽžŠś‚Ģ‚±‚Ę‚ą‚ ‚é‚ŖCœ‘‚Å‚Ķ”’ŒŒ•a‚ŖÄ”­‚µ‚Ä‚¢‚Č‚¢Š°‰šŠś‚É”­Ē‚·‚邱‚Ę‚Ŗ‘½‚¢B‚»‚Ģ‚ꂤ‚ȏĒ—į‚Å‚ąC’†•_ŒoŒn”’ŒŒ•a‚Ģ”­ĒŒćC”ƒJŒŽˆČ“ą‚ɍœ‘Ä”­‚Ŗ”F‚ß‚ē‚ź‚éB’†•_ŒoŒn”’ŒŒ•a‚ĢAML ‚Å‚Ģ”­Ē•p“x‚Ķ5“ˆČ‰ŗ‚Ę•ń‚³‚ź1j`4jCŠ°‰šŒć—Ć–@‚Ę‚µ‚āCAraC ‘å—Ź—Ć–@‚ā“ÆŽķ‘¢ŒŒŠ²×–EˆŚA‚Ŗs‚ķ‚ź‚½ź‡‚ĶC‚»‚Ģ”­Ē•p“x‚Ķ‚³‚ē‚É’į‰ŗ‚·‚邱‚Ę‚ŖŽ¦‚³‚ź‚Ä‚¢‚é4jB‚»‚Ģ’†•_ŒoŒn”’ŒŒ•a‚Ģ—\–hŽ”—Ć‚Ģ‘O•ūŽ‹“IŒ¤‹†‚Ķ1986 ”N‚Ģthe Medical Research CounciliMRCj1j‚©‚ē•ń‚³‚ź‚½‚ą‚Ģ‚Ę1992 ”Nthe Southwest Oncology GroupiSWOGj5j‚©‚ē‚Ģ2 •ń‚Ģ‚Ż‚Å‚ ‚éB‘OŽŅ‚Å‚ĶAraC ‚ĘMTXCŒćŽŅ‚Å‚ĶAraC ‚Ģ‘o“ą“Š—^‚Ŗs‚ķ‚ź‚½‚ŖC‚»‚Ģ—LŒų«‚Ķ–¾‚ē‚©‚Å‚Ķ‚Č‚©‚Į‚½B‚±‚Ģ‚ꂤ‚ÉAML ‚Å‚Ķ’†•_ŒoŒn”’ŒŒ•a‚Ģ”­Ē•p“x‚Ķ’į‚­C—\–hŽ”—Ć‚Ģ—LŒų«‚ąŠm”F‚³‚ź‚Ä‚¢‚Č‚¢BNCCN ƒKƒCƒhƒ‰ƒCƒ“‚Å‚ą’†•_ŒoŒn”’ŒŒ•a‚Ģ—\–hŽ”—Ć‚Ķ„§‚µ‚Ä‚¢‚Č‚¢‚ŖC”­Ē•p“x‚Ģ‚‚¢CFAB •Ŗ—Ž‚Å‚ĢM4CM5Cbiphenotype ‚ā––½”’ŒŒ‹…”‚Ŗ10 –œ/ƒŹL ‚š’“‚¦‚éĒ—į‚Ķ—įŠO‚Ę‚µ‚Ä‚¢‚éB

’†•_ŒoŒn”’ŒŒ•a‚Ŗ”­Ē‚µ‚½ź‡‚ĢŽ”—Ć‚ĶCEuropean LeukemiaNet ‚Å‚ĶAraC 40`50 mg ‚ĢT2`3 ‰ń‘o“ą“Š—^‚Ŗ„§‚³‚ź‚Ä‚¢‚éB‚Ü‚½C–ņÜ«‚Ģ“ī–Œ‰Š‚š—\–h‚·‚邽‚߃XƒeƒƒCƒh‚Ģ“Š—^‚ą‹LŚ‚³‚ź‚Ä‚¢‚éB

yŽQl•¶Œ£z

1j Rees JK, et al. Principal results of the medical research councilfs 8th acute myeloid leukemia trial. Lancet. 1986 ; 2i8518j : 1236-41.i1iiDj

2j Castagnola C, et al. The value of combination therapy in adult acute myeloid leukemia with central nervous system involvement. Haematokogica. 1997 ; 82i5j : 577-80.i3iDj

3j Shihadeh F, et al. Cytogenetic profile of patients with acute myeloid leukemia and central nervous system disease. Cancer. 2012 ; 118i1j : 112-7.i3iiiDj

4j MartÍnez-Cuadrón D, et al. Central nervous system involvement at first relapse in patients with acute myeloid leukemia. Haematologica. 2011 ; 96i9j : 1375-9.i3iDj

5j Morrison FS, et al. Late intensification with POMP chemotherapy prolongs survival in acute myelogenous leukemia-Results of a Southwest Oncology Group study of rubidazone versus adreamycin for remission induction, prophylactic intrathecal therapy late intensification, and levamisole maintenance. Leukemia. 1992 ; 6i7j : 708-14.i1iiDj


CQ15

ŽīįŽŒ`¬«AML ‚É‘Ī‚µ‚Ä’Źķ‚ĢŠ°‰š“±“ü—Ć–@‚šs‚¤‚Ģ‚Ķ‘Ć“–‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2B
ŽīįŽŒ`¬«AML ‚É‘Ī‚µ‚Ä‚Ķ’P“Ę”­Ē‚Ģź‡‚Å‚ ‚Į‚Ä‚ą’Źķ‚ĢŠ°‰š“±“ü—Ć–@‚Ŗl—¶‚³‚ź‚éB

y‰š ąz

ŽīįŽŒ`¬«AML ‚ĢŽīįŽ‚Ķœ‘‹…Œn‚Ģ”’ŒŒ•a×–E‚É‚ę‚č\¬‚³‚źC—ĪF‚š’ę‚·‚邱‚Ę‚©‚ē—ĪFŽķichloromaj‚ʏĢ‚³‚źCmyeloblastomaCmonoblastoma ‚Ę‚ą‹Lq‚³‚ź‚Ä‚¢‚½‚ŖCWHO •Ŗ—Ž‚Å‚Ķœ‘“÷Žīimyeloid sarcomaj‚Ę‹LŚ‚³‚ź‚Ä‚¢‚éBŽīįŽŒ`¬«AML ‚Ģ”­Ē•p“x‚Ķ3“’ö“x1j‚ŁCŽīįŽ‚ĶC”ē•†CƒŠƒ“ƒpßCĮ‰»ŠĒCœCt‘ŸC“ī•”‘gDC_Œo‘gDC—‘‘ƒCø‘ƒ‚Č‚Ē‘Ģ‚Ģ‚ ‚ē‚ä‚é•”ˆŹ‚ɏoŒ»‚·‚éBŽīįŽ‚ĢoŒ»‚Ķ”’ŒŒ•a‚Ģ”­ĒŽž‚Ģ‚±‚Ę‚Ŗ‘½‚¢‚ŖC”’ŒŒ•a‚Ģ”­Ē‚āÄ”­‚Ģ‘O‹ģĒó‚Ę‚µ‚ďoŒ»‚·‚邱‚Ę‚ą‚ ‚éB‚Ü‚½Cœ‘ˆŁŒ`¬ĒŒóŒQimyelodysplastic syndromesFMDSjCœ‘‘B«Žīį‡imyeloproliferative neoplasmsFMPNj‚ ‚é‚¢‚ĶMDS/MPN ‚Ģ‹}«“]‰»Žž‚É‚ą”F‚ß‚ē‚ź‚éB

ŽīįŽŒ`¬«AML ‚Ķ”­Ē•p“x‚ą’į‚¢‚±‚Ę‚©‚ēCŽ”—Ć–@‚É‚Ā‚¢‚Ä‚Ģ‘O•ūŽ‹“IŒ¤‹†‚Ķs‚ķ‚ź‚Ä‚Ø‚ē‚øCĒ—į•ń‚šŽę‚č‚Ü‚Ę‚ß‚½‚ą‚Ģ‚Ŗ‚Ł‚Ę‚ń‚Ē‚Å‚ ‚éB1986 ”N‚ÉEshghabadi ‚ē‚Ķ1 —į‚ĢŽ©Œ±—į‚É•¶Œ£lŽ@‚š‰Į‚¦‚Ä•¹—p‰»Šw—Ć–@‚šŠ©‚ß‚Ä‚¢‚é2jB2002 ”N‚É‚ĶŽR“ą‚ĘˆĄ“c‚ŖC2 —į‚ĢŽ©Œ±—į‚šŠÜ‚ß‚½74 —į‚ĢŽīįŽŒ`¬«AML ‚ĢŽ”—Ɛ¬Ń‚šŒŸ“¢‚µC‰»Šw—Ć–@‚Ģ—LŒų«‚š•ń‚µ‚Ä‚¢‚é3jBMD ƒAƒ“ƒ_[ƒ\ƒ“‚Ŗ‚ńƒZƒ“ƒ^[‚Å‚ĢŒ¤‹†‚Å‚ĶCŽīįŽŒ`¬«AML 23 —į‚Ģ‚¤‚æ’Źķ‚Ģ‰»Šw—Ć–@‚šŽó‚Æ‚½16 —į‚Ę’Źķ‚ĢAML 1,720 —į‚Ę‚š”äŠr‚µCŠ°‰š—¦C2 ”N‚Ģ–³ƒCƒxƒ“ƒg¶‘¶Š„‡iEFSjC‘S¶‘¶Š„‡iOSj‚ɍ·‚Ŗ‚Č‚©‚Į‚½‚±‚Ę‚šŽ¦‚µC”’ŒŒ•a‚Ģ”­Ē‚ɐęs‚µ‚½ŽīįŽŒ`¬«AML ‚É‘Ī‚·‚鑁Šś‚Ģ‰»Šw—Ć–@‚ĢŽĄŽ{‚šŠ©‚ß‚Ä‚¢‚é4jB‚Ü‚½CNCCN ƒKƒCƒhƒ‰ƒCƒ“‚āEuropean LeukemiaNet ‚É‚Ø‚¢‚Ä‚ąŽīįŽŒ`¬«AML ‚É‘Ī‚µ‚ĉ»Šw—Ć–@‚Ŗ„§‚³‚ź‚Ä‚¢‚éB

yŽQl•¶Œ£z

1j Muss HB, et al. Chloroma and other myeloblastic tumors. Blood. 1973 ; 42i5j : 721-8.i3iDj

2j Eshghabadi M, et al. Isolated granulocytic sarcoma : report of a case and review of the literature. J Clin Oncol. 1986 ; 4i6j : 912-7.i3iiiDj

3j Yamauchi K, et al. Comparison in treatments of nonleukemic granulocytic sarcoma : report of two cases and a review of 72 cases in the literature. Cancer. 2002 ; 94i6j : 1739-46.i3iiiDj

4j Tsimberidou AM, et al. Myeloid sarcoma is associated with superior event-free survival and overall survival compared with acute myeloid leukemia. Cancer. 2008 ; 113i6j : 1370-8.i3iiiDj


2
  ‹}«‘Oœ‘‹…«”’ŒŒ•a
iacute promylocytic leukemiaFAPLj

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1D•aˆöE•a‘Ō

‹}«‘Oœ‘‹…«”’ŒŒ•aiacute promylocytic leukemiaFAPLj‚ĶCFAB •Ŗ—Ž‚Å‚ĶM3CWHO •Ŗ—Ži2008j‚Å‚Ķ”½•œ«õF‘ĢˆŁķt (15;17)(q22;q12)GPML-RARA ‚š”ŗ‚¤‹}«‘Oœ‘‹…«”’ŒŒ•aC‚Ģ–¼Ģ‚ŌĂĪ‚ź‚Ä‚¢‚éBAML ‚Ģ10`15“‚šč‚ß‚Ä‚Ø‚čC30`50 Ī‘ä‚ĢŽį”N‘w‚ɍD”­‚µ60 ĪˆČć‚ÅŒø­ŒXŒü‚Ę‚Č‚é1jBAPL ‚ĢŽ”—Ć‚ĶCPML-RARA —Z‡ˆā“`Žq‚ɍģ—p‚·‚éCƒI[ƒ‹ƒgƒ‰ƒ“ƒXŒ^ƒŒƒ`ƒmƒCƒ“Ž_iall-trans retinoic acidFATRAj‚ĘˆŸƒqŽ_iarsenic trioxideFATOj‚Ģ—LŒų«‚ŖŠm—§‚³‚ź‚Ä‚¢‚é“_‚Ŗ‘¼‚Ģ‹}«”’ŒŒ•a‚Ę‘å‚«‚­ˆŁ‚Č‚Į‚Ä‚¢‚éBAPL ‚Ķ”­•a‰Šś‚ɐü—n˜“iŒ^‚Ģ”dŽķ«ŒŒŠĒ“ą‹ĆŒÅĒŒóŒQidisseminated intravascular coagulationFDICj‚Ŗ‚Ż‚ē‚ź’v–½“I‚Č‘ŸŠķoŒŒ‚š‡•¹‚µ‚ā‚·‚¢‚ŖCDIC ‚šƒRƒ“ƒgƒ[ƒ‹‚·‚é‚ĘŠ®‘SŠ°‰š—¦‚Ķ80`90“ˆČćC–³•a¶‘¶Š„‡iDFSj‚Ķ60`80“ˆČć‚Å‚ ‚čC‘¼‚ĢAML ‚Ę”äŠr‚µ‚č‚‚¢Ž”–ü—¦‚Ŗ“¾‚ē‚ź‚é2jB‚±‚Ģ‚ꂤ‚ɁCAPL ‚ĶŽ¾Š³“ĮˆŁ“I‚Č•ŖŽq‚ɍģ—p‚·‚é•ŖŽq•W“I—Ć–@‚Ŗ—LŒų‚Č”’ŒŒ•a‚Å‚ ‚éB

2D—\ŒćˆöŽq2j

—\ŒćˆöŽq‚É‚ę‚é‘w•Ź‰»‚Ę‚µ‚Ä’įƒŠƒXƒNi”’ŒŒ‹……10,000/ƒŹLjC‚ƒŠƒXƒNi”’ŒŒ‹…„10,000/ƒŹLj‚Ģ2 ŒQ•Ŗ—Ž‚ŖL‚­—p‚¢‚ē‚ź‚Ä‚¢‚é‚Ł‚©CŒŒ¬”Ā”‚š‰Į‚¦‚½3 ŒQ•Ŗ—ŽC’įƒŠƒXƒNi”’ŒŒ‹……10,000/ƒŹLCŒŒ¬”Ā„40,000/ƒŹLjC’†ƒŠƒXƒNi”’ŒŒ‹……10,000/ƒŹLCŒŒ¬”Ā…40,000/ƒŹLjC‚ƒŠƒXƒNi”’ŒŒ‹…„10,000/ƒŹLj‚ą—p‚¢‚ē‚ź‚Ä‚¢‚éB

3DŠ°‰š“±“ü—Ć–@3j4j

ATRA ‚ĘƒAƒ“ƒgƒ‰ƒTƒCƒNƒŠƒ“Œn–ņÜmƒCƒ_ƒ‹ƒrƒVƒ“iIDRj‚Ü‚½‚Ķƒ_ƒEƒmƒ‹ƒrƒVƒ“iDNRjn‚ĘƒVƒ^ƒ‰ƒrƒ“iAraCj‚Ģ•¹—p—Ć–@‚ŖŠī–{‚Å‚ ‚čCŠ°‰š“±“ü—¦‚ĶŠT‚Ė90`95“‚Å‚ ‚éBDIC ‚É‘Ī‚·‚é\•Ŗ‚ČŽ”—Ć‚šs‚¤BAPL ‚Ŗ—Տ°“ICŒ`‘ŌŠw“I‚É‹^‚ķ‚ź‚鏼‡‚Ķ‰Ā‹y“I‘¬‚ā‚©‚ÉATRA “Š—^‚šŠJŽn‚µCt (15;17) ‚āPML-RARA ‚ĢŒŸøŒ‹‰Ź‚É‚ę‚éŠm’čf’f‚š‘Ņ‚Ā‚±‚Ę‚ŖoŒŒ‡•¹Ē‚Ģ—\–h‚É—LŒų‚Å‚ ‚éBATRA “Š—^’†‚ĶAPL •Ŗ‰»ĒŒóŒQidifferentiation syndromeFDSj‚Ģ‡•¹‚É’ˆÓ‚·‚éiCQ4jB

4D’nŒÅ‚ß—Ć–@3j4j

Š°‰š“±“ü—Ć–@‚Ę“Æ‚¶‚­ƒAƒ“ƒgƒ‰ƒTƒCƒNƒŠƒ“Œn–ņÜ‚ĘAraC ‚ŖŠī–{‚Å‚ ‚čC2 ‚Č‚¢‚µ3 ƒR[ƒXs‚ķ‚ź‚éB‚Č‚ØAraC ‚Ģ•¹—p‚Ķ‚ƒŠƒXƒNŒQ‚Å‚Ģ‚Ż—L—pC‚Ü‚½’nŒÅ‚ß—Ć–@‚É‚Ø‚¢‚Ä‚ąATRA ‚Ģ•¹—p‚Ŗ—LŒų‚Å‚ ‚é‚Ę‚·‚é•ń‚ą‚ ‚éBŽĄ’n—Տ°‚Å‚Ķ‚¢‚ø‚ź‚Ģ•ū–@‚š—p‚¢‚Ä‚ą–ā‘č‚Č‚¢BÅ‚ąd—v‚Čƒ|ƒCƒ“ƒg‚Ķ’nŒÅ‚ß—Ć–@I—¹Žž‚É•ŖŽq¶•ØŠw“IŠ°‰š‚Ŗ’B¬‚³‚ź‚Ä‚¢‚邱‚Ę‚Å‚ ‚éB

5DˆŪŽ—Ć–@‚ĘŒo‰ßŠĻŽ@3j4j

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6DÄ”­APL ‚ĢŽ”—Ć3j4j

ATRA ‚ʉ»Šw—Ć–@‚É‚ę‚鎔—ĆŒć‚ĢÄ”­APL ‚É‘Ī‚µCATO ‚É‚ę‚éÄŠ®‘SŠ°‰š—¦‚Ķ80`90“‚Ę‚«‚ķ‚߂č‚‚­CATO ’P“Ę‚Å•ŖŽq¶•ØŠw“IŠ°‰š‚š“¾‚ē‚ź‚邱‚Ę‚Ŗ“Į•M‚·‚ׂ«“Į’„‚Å‚ ‚éB‚Ü‚½‘ę“ń‘I‘š–ņ‚Ę‚µ‚ă^ƒ~ƒoƒƒeƒ“‚ą—LŒų‚Å‚ ‚éB

7D”Œć‚Ģ“W–]4j

APL ‚ĶATRA ‚š—p‚¢‚½•W€Ž”—Ć‚Ŗ‚Ł‚ŚŠm—§‚³‚ź‚Ä‚¢‚éB‚µ‚©‚µ‚Č‚Ŗ‚ēŽ”—Ə‰Šś‚É‚Ż‚ē‚ź‚éDIC ‘Īō‚ĶˆĖ‘R‚Ę‚µ‚ċɂ߂ďd—v‚Å‚ ‚éB‹ß”NATO ‚āƒ^ƒ~ƒoƒƒeƒ“‚É‚ę‚čÄ”­—į‚É‚Ø‚¢‚Ä‚ą‚‚¢Š°‰š—¦‚Ŗ“¾‚ē‚ź‚é‚ꂤ‚É‚Č‚čCŒ»Ż‚±‚ź‚ē‚Ģ–ņÜ‚š‰”­—į‚É‘Ī‚µ‚Ä—p‚¢‚½Œ¤‹†‚Ŗis’†‚Å‚ ‚éB

yŽQl•¶Œ£z

1j Swerdlow SH, et al. eds. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon, IARC ; 2008 ; pp112-4.

2j Sanz MA, et al. Definition of relapse risk and role of nonanthracycline drugs for consolidation in patients with acute promyelocytic leukemia : a joint study of the PETHEMA and GIMEMA cooperative groups. Blood. 2000 ; 96i4j : 1247-53.i3iiiDiij

3j Sanz MA, et al. Management of acute promyelocytic leukemia : recommendations from an expert panel on behalf of the European Leukemia Net. Blood. 2009 ; 113i9j : 1875-91.iƒKƒCƒhƒ‰ƒCƒ“j

4j Sanz MA, et al. Modern Approaches to Treating Acute Promyelocytic Leukemia. J Clin Oncol. 2011 ; 29i5j : 495-503.iƒŒƒrƒ…[j



ŸƒAƒ‹ƒSƒŠƒYƒ€

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‹}«‘Oœ‘‹…«”’ŒŒ•a‚ĢƒAƒ‹ƒSƒŠƒYƒ€ CQ1 CQ2 CQ3 CQ4 CQ5 CQ6 CQ7 CQ8

APL ‚Ģf’fiCQ1j‚Å‚ĶFISH ‚āRT-PCR –@‚É‚ę‚éPML-RARA ‚ĢŒŸo‚Ŗd—v‚Å‚ ‚éBt (15 ;17) ˆČŠO‚Ģ“]Ą‚Å‚ĶƒI[ƒ‹ƒgƒ‰ƒ“ƒXŒ^ƒŒƒ`ƒmƒCƒ“Ž_iATRAj‚āˆŸƒqŽ_iATOj‚Ģ”½‰ž«‚ŖˆŁ‚Č‚é‚©‚ē‚Å‚ ‚éBAPL ‚Ģ‰ŠśŽ”—Ć‚É‚Ø‚¢‚Ä‚Ķ‹ĆŒÅˆŁķ‚É”ŗ‚¤”]oŒŒ‚Ę”xoŒŒ‚É‚ę‚鑁ŠśŽ€–S‚Ŗ”ńŠ°‰š‚ĢŽåˆö‚Ę‚Č‚é‚Ģ‚Å‹ĆŒÅŒŸø‚š•p‰ń‚ɍs‚¤•K—v‚Ŗ‚ ‚éBAPL ‚Ģ–³•a¶‘¶Š„‡iDFSj‚É‚Ø‚Æ‚é—\Œć•s—ĒˆöŽq‚ĶŽ”—Ć‘O”’ŒŒ‹…”10,000/ƒŹL ˆČć‚Å‚ ‚éB

–¢Ž”—ĆAPL ‚ĢŠ°‰š“±“ü—Ć–@iCQ2j‚Å‚ĶATRA ‚ʉ»Šw—Ć–@‚Ģ•¹—p‚Ŗ•W€—Ć–@‚Å‚ ‚éB‰‰ńŠ°‰š“±“ü—Ć–@‚Å‚ĶŽ”—Ć’ļR—į‚Ķ‚Ł‚Ę‚ń‚Ē‚Č‚­CoŒŒ‚ĘAPL •Ŗ‰»ĒŒóŒQ‚Č‚Ē‚É‚ę‚鑁ŠśŽ€–S‚Ŗ”ńŠ°‰š‚ĢŽåˆö‚Å‚ ‚éB‚µ‚½‚Ŗ‚Į‚āCoŒŒ—\–hiCQ3j‚ĘAPL •Ŗ‰»ĒŒóŒQ‘ĪōiCQ4j‚Ŗd—v‚Å‚ ‚éB

ŒŒ‰tŠw“IŠ°‰š‚Ŗ“¾‚ē‚ź‚½ŒćC2 ‚Č‚¢‚µ3 ƒR[ƒX‚Ģ‰»Šw—Ć–@‚©‚ē‚Č‚é’nŒÅ‚ß—Ć–@iCQ5j‚šs‚¢CRQ-PCR –@‚š—p‚¢‚½œ‘×–E‚ĢPML-RARA ‰A«‰»‚É‚ę‚é•ŖŽq¶•ØŠw“IŠ°‰š‚Ö‚Ģ“ž’B‚š–ŚŽw‚·B’nŒÅ‚ß—Ć–@‚É‚Ø‚Æ‚éATRA ‚Ģ•¹—p‚āATO ‚Ģ“±“ü‚ŖŽŽ‚Ż‚ē‚ź‚Ä‚¢‚éB

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‚»‚Ģ‘¼C“ĮŽź‚ČAPL ‚ĢŽ”—Ć‚Ę‚µ‚č‚—īŽŅiCQ9j‚Ę”D•wiCQ10j‚ĢŽ”—Ć‚É‚Ā‚¢‚Ä‚ą‹Lq‚·‚éB



CQ1

‰”­APL ‚ĢŽ”—ĆŠJŽn‘O‚ɍs‚¤‚ׂ«ŒŸø‚Ę—\ŒćˆöŽq‚Ķ‰½‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2A
FISH ‚Č‚¢‚µ‚ĶRT-PCR ‚É‚ę‚éPML-RARA ‚Ģ‘Šśf’f‚ŖŠ©‚ß‚ē‚ź‚éB

„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[1
—\ŒćˆöŽq‚Å‚ ‚鎔—Ć‘O”’ŒŒ‹…”‚É‚ę‚莔—Ɛķ—Ŗ‚š—§‚Ă邱‚Ę‚ŖŠ©‚ß‚ē‚ź‚éB

„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2B
‘ŸŠķoŒŒ‚É‚ę‚鑁ŠśŽ€–S‚Ģ—\–h‚Ģ‚½‚ß‚É•p‰ń‚Ģ‹ĆŒÅŒŸø‚ŖŠ©‚ß‚ē‚ź‚éB

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APL ‚Ģ–ń98“‚Ét (15;17) —R—ˆ‚ĢPML-RARA ‚Ŗ—z«‚Å‚ ‚é1jBŽc‚鏭”—į‚Ģ‘å•”•Ŗ‚ąRARA ‚É“]Ą‚·‚é‚ŖCƒI[ƒ‹ƒgƒ‰ƒ“ƒXŒ^ƒŒƒ`ƒmƒCƒ“Ž_iATRAj‚Ø‚ę‚ŃˆŸƒqŽ_iATOj‚É‘Ī‚·‚锽‰ž«‚ŖˆŁ‚Č‚é1jB—į‚¦‚ĪCt (11;17) —R—ˆ‚ĢPLZF-RARA —z«—į‚É‚Ķ‚Ę‚ą‚É–³Œų‚Å‚ ‚éB‚µ‚½‚Ŗ‚Į‚āCATRA ‚ʉ»Šw—Ć–@‚Ģ•¹—p‚É‚ę‚鏉ŠśŽ”—Ć‚Ŗ•W€—Ć–@‚Å‚ ‚éAPL ‚É‚Ø‚¢‚Ä‚ĶFISH ‚āRT-PCR –@‚É‚ę‚čPML-RARA ‚Ŗ—z«‚©”Ū‚©‚š‘Šś‚ɐf’f‚·‚邱‚Ę‚Ŗd—v‚Å‚ ‚éB‚Ü‚½CPML-RARA ‚Ģ’č—Ź‚Ķ”÷¬Žc‘¶•a•Ļ‚ĢŒŸo‚É—L—p‚Å‚ ‚čC’nŒÅ‚ß—Ć–@Œć‚Ģ—L–³‚Ķd—v‚Č—\ŒćˆöŽq‚Ę‚Č‚čCŽ”—Ć•ūj‚š¶‰E‚·‚é‚Ģ‚Őf’fŽž‚É‚»‚Ģ—L–³‚šŠm”F‚·‚é•K—v‚Ŗ‚ ‚é2jB

č÷—±‚Ŗ–R‚µ‚¢M3v ‚Ģf’f‚Ķ‚Ę‚«‚ɍ¢“ļ‚Å‚ ‚éBM3v ‚Ķ”’ŒŒ‹…‚’l—į‚É‘½‚­CCD2 —z«‚Ę‘ŠŠÖ‚·‚éBM3v ‚Ģ‘S¶‘¶Š„‡iOSjC–³•a¶‘¶Š„‡iDFSj‚ĶM3 ‚Ę”äŠr‚·‚é‚Ę—LˆÓ‚É’į‚¢‚ŖC”’ŒŒ‹…”‚Č‚Ē‚Å•ā³‚·‚é‚ĘM3v ‚Ģ‚Ż‚Å‚Ķ—LˆÓ·‚Ŗ‚Č‚­‚Č‚é3jB

APL ‚É‚Ø‚Æ‚éDFS ‚É‚Ø‚Æ‚é—\Œć•s—ĒˆöŽq‚ĶŽ”—Ć‘O”’ŒŒ‹…”10,000/ƒŹL ˆČć‚Å‚ ‚é4j5jB‚Ü‚½CCD56 —z«‚Ķ–ń10“‚É‚Ż‚ē‚źC”’ŒŒ‹…”‚’lCPML Ų’f“_bcr3 ‚Ę‘ŠŠÖ‚·‚éBCD56 —z«—į‚Ķ‘ŠOÄ”­‚ĢƒŠƒXƒN‚ą‚‚­CÄ”­‚É‘Ī‚·‚é“Ę—§‚µ‚½—\Œć•s—ĒˆöŽq‚Å‚ ‚é6jBFLT3 ˆā“`Žq•ĻˆŁ‚ĶAPL ‚Ģ–ń40“‚É‚Ż‚ē‚źCŽ”—Ć‘O”’ŒŒ‹…”CM3v ‚ābcr3 ‚Ę‘ŠŠÖ‚·‚éB‘½”—į‚Ģ‰šĶ‚Å‚Ķ”ńŠ°‰š‚Ę‘ŠŠÖ‚µC5 ”NÄ”­—¦‚āOS ‚Ę‚Ķ‘ŠŠÖ‚µ‚Č‚©‚Į‚½7jB

ATRA ‚ʉ»Šw—Ć–@‚É‚ę‚鏉ŠśŽ”—Ć‚É‘Ī‚·‚鎔—Ć’ļR—į‚Ķ‚Ł‚Ę‚ń‚Ē‚Č‚­CoŒŒ‚ĘAPL •Ŗ‰»ĒŒóŒQ‚Č‚Ē‚É‚ę‚鑁ŠśŽ€–S‚Ŗ–ā‘č‚Å‚ ‚éBAPL •Ŗ‰»ĒŒóŒQ‚š—\‘Ŗ‚·‚éŽw•W‚Ķ‚Č‚¢‚ŖC‹ĆŒÅˆŁķ‚š•p‰ń‚Ƀ`ƒFƒbƒN‚µ‚ďoŒŒ‚Ģ—\–h‚šs‚¤‚±‚Ę‚Ŗd—v‚Å‚ ‚éiCQ3CCQ4jB

yŽQl•¶Œ£z

1j Grimwade D, et al. Characterization of acute promyelocytic leukemia cases lacking the classic t (15;17) : results of the European Working Party. Blood. 2000 ; 96i4j : 1297-308.i3iDivj

2j Grimwade D, et al. Prospective minimal residual disease monitoring to predict relapse of acute promyelocytic leukemia and to direct pre-emptive arsenic trioxide therapy. J Clin Oncol. 2009 ; 27 i22j : 3650-8.i3iDiij

3j Tallman MS, et al. Does microgranular variant morphology of acute promyelocytic leukemia independently predict a less favorable outcome compared with classical M3 APL? A joint study of the North American Intergroup and the PETHEMA Group. Blood. 2010 ; 116i25j : 5650-9.i3iAj

4j Asou N, et al. Analysis of prognostic factors in newly diagnosed acute promyelocytic leukemia treated with all-trans retinoic acid and chemotherapy. Japan Adult Leukemia Study Group. J Clin Oncol. 1998 ; 16i1j : 78-85.i3iDiij

5j Sanz MA, et al. Definition of relapse risk and role of nonanthracycline drugs for consolidation in patients with acute promyelocytic leukemia : a joint study of the PETHEMA and GIMEMA cooperative groups. Blood. 2000 ; 96i4j : 1247-53.i3iiiDiij

6j Montesinos P, et al. Clinical significance of CD56 expression in patients with acute promyelocytic leukemia treated with all-trans retinoic acid and anthracycline-based regimens. Blood. 2011 ; 117 i6j : 1799-805.i3iDij

7j Gale RE, et al. Relationship between FLT3 mutation status, biologic characteristics, and response to targeted therapy in acute promyelocytic leukemia. Blood. 2005 ; 106i12j : 3768-76.i3iAj


CQ2

‰”­APL ‚ĢŠ°‰š“±“ü—Ć–@‚Ę‚µ‚ĉ½‚ŖŠ©‚ß‚ē‚ź‚é‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[1
‰”­APL ‚Ģ‰‰ńŠ°‰š“±“ü—Ć–@‚Ę‚µ‚āCATRA ‚ĘƒAƒ“ƒgƒ‰ƒTƒCƒNƒŠƒ“Œn‚šŽå‘Ģ‚Ę‚µ‚½‰»Šw—Ć–@‚Ģ•¹—p‚ŖŠ©‚ß‚ē‚ź‚éB

„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2B
–¢Ž”—ĆAPL ‚Ģ‰‰ńŠ°‰š“±“ü—Ć–@‚É‚Ø‚¢‚āCATRA ‚ʉ»Šw—Ć–@‚ɉĮ‚¦‚ĈŸƒqŽ_‚š—p‚¢‚鎔—Ć‚Ķ–³•a¶‘¶Š„‡‚š‰ü‘P‚·‚é‰Ā”\«‚Ŗ‚ ‚éi‘“ą“K‰žŠOjB

y‰š ąz

‰”­APL —į‚É‘Ī‚·‚鏉‰ńŠ°‰š“±“ü—Ć–@‚É‚Ø‚¢‚āC‰»Šw—Ć–@‚Ģ‚Ż‚ĢŒQ‚ʉ»Šw—Ć–@‚ɃI[ƒ‹ƒgƒ‰ƒ“ƒXŒ^ƒŒƒ`ƒmƒCƒ“Ž_iATRAj‚š‰Į‚¦‚½ŒQ‚Ģ‘å‹K–Ķƒ‰ƒ“ƒ_ƒ€‰»”äŠrŽŽŒ±‚Ŗƒˆ[ƒƒbƒp‚Ę•Ä‘‚ōs‚ķ‚źC‚Ę‚ą‚ÉŠ°‰š—¦‚É‚Ķ—LˆÓ·‚š”F‚ß‚Č‚©‚Į‚½‚ŖC–³•a¶‘¶Š„‡iDFSj‚ĶATRA •¹—pŒQ‚Ŗ—LˆÓ‚É—ĒD‚Å‚ ‚Į‚½1j2jB‚ķ‚Ŗ‘‚Å‚ąJapan Adult Leukemia Study GroupiJALSGj APL92 Œ¤‹†‚É‚Ø‚¢‚ÄATRA ‚ʉ»Šw—Ć–@‚Ģ•¹—p‚šs‚¢C‰»Šw—Ć–@‚Ģ‚Ż‚ĢˆČ‘O‚ĢAML87CAML89 Œ¤‹†‚Ę”äŠr‚µ‚Ä—D‚ź‚½–³ƒCƒxƒ“ƒg¶‘¶Š„‡iEFSj‚Ŗ“¾‚ē‚ź‚½3jB

ATRA ‚É•¹—p‚·‚鉻Šw—Ć–@‚Ģ“ą—e‚É‚Ā‚¢‚Ä‚Ķ\•Ŗ‚ČƒGƒrƒfƒ“ƒX‚Ķ‚Č‚¢BƒAƒ“ƒgƒ‰ƒTƒCƒNƒŠƒ“Œn‚Ģ—LŒų«‚Ŗ‚‚­C‚¢‚ø‚ź‚ĢŽ”—Ć–@‚É‚Ø‚¢‚Ä‚ą—p‚¢‚ē‚ź‚é4jB60 Ī–¢–ž‚ĢŽ”—Ć‘O”’ŒŒ‹…”10,000/ƒŹL ˆČ‰ŗ‚š‘ĪŪ‚ɁCƒAƒ“ƒgƒ‰ƒTƒCƒNƒŠƒ“Œn‚ɉĮ‚¦‚ăVƒ^ƒ‰ƒrƒ“iAraCj‚Ģ—L–³‚É‚ę‚é”äŠrŽŽŒ±‚šs‚Į‚½ƒˆ[ƒƒbƒpAPL2000 Œ¤‹†‚Å‚Ķ2 ”N—ŻĻÄ”­—¦icumulative incidence of relapseFCIRjCEFSC‘S¶‘¶Š„‡iOSj‚¢‚ø‚ź‚ąAraC •¹—pŒQ‚Ŗ—LˆÓ‚É—D‚ź‚Ä‚¢‚½5jB‚µ‚©‚µ‚Č‚Ŗ‚ēC—¼ŒQ‚ĢƒAƒ“ƒgƒ‰ƒTƒCƒNƒŠƒ“‚Ķ“Æ—Ź‚Å‚ ‚čCAraC •¹—p‚Ģ•Ŗ‚¾‚ÆŽ”—Ć‹­“x‚Ŗ‹­‚©‚Į‚½‚ʍl‚¦‚ē‚ź‚éBƒAƒ“ƒgƒ‰ƒTƒCƒNƒŠƒ“Œn‚Ģ‚Ż‚š‰»Šw—Ć–@‚É—p‚¢‚éPETHEMA Œ¤‹†‚ĘAPL2000 Œ¤‹†‚ĢŒ‹‰Ź‚š”äŠr‚·‚é‚ʁC”’ŒŒ‹…”10,000/ƒŹL ˆČ‰ŗ‚ĢĒ—į‚Å‚ĶPETHEMA Ž”—Ć‚Ģ•ū‚Ŗ3 ”NCIR ‚Ķ—LˆÓ‚É’į‚©‚Į‚½6jBˆź•ūC”’ŒŒ‹…”10,000/ƒŹL ˆČć‚ĢĒ—į‚Å‚ĶAraC ‚š•¹—p‚µ‚½APL2000 ‚ĢOS ‚Ŗ—ĒD‚ÅCIR ‚ą’į‚¢ŒXŒü‚É‚ ‚Į‚½B”’ŒŒ‹…”’į’l—į‚Ģ‰»Šw—Ć–@‚Ģ•¹—pŽžŠś‚ąˆÓŒ©‚Ŗ•Ŗ‚©‚ź‚éBƒˆ[ƒƒbƒpAPL93 Œ¤‹†‚Å‚Ķ”’ŒŒ‹…”5,000/ƒŹL ˆČ‰ŗ‚š‘ĪŪ‚ɍŏ‰‚©‚ē‰»Šw—Ć–@‚š•¹—p‚·‚éŒQ‚Ę”’ŒŒ‹…”‚ɉž‚¶‚ĉ»Šw—Ć–@‚š’Ē‰Į‚·‚éŒQ‚Ģ”äŠrŽŽŒ±‚Ŗs‚ķ‚źC2 ”NOS ‚ɍ·‚Ķ‚Č‚¢‚ŖC‘OŽŅ‚ĢCIR ‚Ŗ—LˆÓ‚É’į‚©‚Į‚½7jBJALSG ‚Å‚ĶŽ”—Ć‘O”’ŒŒ‹…”‚ɉž‚¶‚Ä•¹—p‚·‚鉻Šw—Ć–@‚š‘w•Ź‰»‚µC”’ŒŒ‹…”3,000/ƒŹL –¢–ž‚©‚ĀAPL ×–E1,000/ƒŹL –¢–ž‚Ģ—į‚Å‚ĶATRA ’P“Ę‚ÅŽ”—Ć‚šŠJŽn‚µ‚Ä”’ŒŒ‹…‘‰Į—į‚ɉ»Šw—Ć–@‚š’Ē‰Į‚µCŠeŒQ‚É‘»F‚Č‚¢¬Ń‚Ŗ“¾‚ē‚ź‚Ä‚¢‚é8jB

Ä”­—į‚É—LŒų‚ČˆŸƒqŽ_iATOj‚š‰‰ńŠ°‰š“±“ü‚É—p‚¢‚½ćŠCƒOƒ‹[ƒv‚ĢŒ¤‹†‚Å‚ĶATRA ’P“ʁCATO ’P“ĘŒQ‚Ę”äŠr‚µ‚Ä—¼ŽŅ‚Ģ•¹—pŒQ‚ĢDFS ‚Ŗ—LˆÓ‚É—ĒD‚Å‚ ‚Į‚½9jB‚Ü‚½CATO ’P“Ę‚ÅŠ°‰š“±“ü‚ĘŠ°‰šŒćŽ”—Ć‚šs‚¤ƒCƒ“ƒhƒOƒ‹[ƒv‚Ģ¬Ń‚Å‚ĶŠ°‰š—¦86“CDFS 80“COS 74“‚Ę—ĒD‚Ȑ¬Ń‚Ŗ“¾‚ē‚źC‰‰ńŠ°‰š“±“ü‚É‚Ø‚Æ‚éATO ‚Ģ“±“ü‚Ķ—LŒų‚ʍl‚¦‚ē‚ź‚é10jB

yŽQl•¶Œ£z

1j Fenaux P, et al. Effect of all transretinoic acid in newly diagnosed acute promyelocytic leukemia. Results of a multicenter randomized trial. European APL 91 Group. Blood. 1993 ; 82i11j : 3241-9.i1iiDiij

2j Tallman MS, et al. All-trans-retinoic acid in acute promyelocytic leukemia. N Engl J Med. 1997 ; 337i15j : 1021-8.i1iiAj

3j Kanamaru A, et al. All-trans retinoic acid for the treatment of newly diagnosed acute promyelocytic leukemia. Japan Adult Leukemia Study Group. Blood. 1995 ; 85i5j : 1202-6.i2Diij

4j Head D, et al. Effect of aggressive daunomycin therapy on survival in acute promyelocytic leukemia. Blood. 1995 ; 86i5j : 1717-28.i2Aj

5j Adès L, et al. Is cytarabine useful in the treatment of acute promyelocytic leukemia? Results of a randomized trial from the European Acute Promyelocytic Leukemia Group. J Clin Oncol. 2006 ; 24i36j : 5703-10. i1iiDij

6j Adès L, et al. Treatment of newly diagnosed acute promyelocytic leukemia iAPLj : a comparison of French-Belgian-Swiss and PETHEMA results. Blood. 2008 ; 111i3j : 1078-84.i3iiiDij

7j Fenaux P, et al. A randomized comparison of all transretinoic acid iATRAj followed by chemotherapy and ATRA plus chemotherapy and the role of maintenance therapy in newly diagnosed acute promyelocytic leukemia. The European APL Group. Blood. 1999 ; 94i4j : 1192-200.i1iiDiij

8j Asou N, et al. A randomized study with or without intensified maintenance chemotherapy in patients with acute promyelocytic leukemia who have become negative for PML-RARalpha transcript after consolidation therapy : the Japan Adult Leukemia Study GroupiJALSGj APL97 study. Blood. 2007 ; 110i1j : 59-66.i1iiDii/3iiiDiij

9j Shen ZX, et al. All-trans retinoic acid/As2O3 combination yields a high quality remission and survival in newly diagnosed acute promyelocytic leukemia. Proc Natl Acad Sci U S A. 2004 ; 101 i15j : 5328-35. i1iiDiij

10j Mathews V, et al. Single-agent arsenic trioxide in the treatment of newly diagnosed acute promyelocytic leukemia : long-term follow-up data. J Clin Oncol. 2010 ; 28i24j : 3866-71.i3iiiAj


CQ3

‰”­APL ‚ĢŠ°‰š“±“ü—Ć–@‚É‚Ø‚Æ‚éDIC ‘Īō‚Ę‚µ‚ĉ½‚ŖŠ©‚ß‚ē‚ź‚é‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2B
‰”­APL ‚ĢATRA ‚ʉ»Šw—Ć–@‚É‚ę‚鏉‰ńŠ°‰š“±“ü—Ć–@‚É‚Ø‚¢‚āC’įƒtƒBƒuƒŠƒmƒQƒ“ŒŒĒiƒ100 mg/dLjC ”’ŒŒ‹…”‚’li„ 20,000/ƒŹLj‚Ø‚ę‚ŃPS 2`3 ‚ĢĒ—į‚ĶoŒŒ‚Ģ‚ƒŠƒXƒNŒQ‚Å‚ ‚čC\•Ŗ‚ȏoŒŒ—\–h‚ŖŠ©‚ß‚ē‚ź‚éB

„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2A
Š°‰š“±“ü—Ć–@’†‚ĢoŒŒ—\–h‚É‚ĶCŒŒ¬”Ā—AŒŒ‚É‚ę‚čŒŒ¬”Ā”30,000`50,000/ƒŹL ˆČćC“€Œ‹ŒŒŸ÷‚É‚ę‚čƒtƒBƒuƒŠƒmƒQƒ“150 mg/dL ˆČć‚É•Ū‚Ā•ā[—Ć–@‚Ŗ‘E‚ß‚ē‚ź‚éB

„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[3
’į•ŖŽqƒwƒpƒŠƒ“Cƒ_ƒiƒoƒƒCƒhƒiƒgƒŠƒEƒ€Cˆā“`Žq‘g‚ŻŠ·‚¦ƒgƒƒ“ƒ{ƒ‚ƒWƒ…ƒŠƒ“‚Č‚Ē‚É‚ę‚éR‹ĆŒÅ—Ć–@‚Ø‚ę‚Ńƒgƒ‰ƒlƒLƒTƒ€Ž_‚É‚ę‚éRü—n—Ć–@‚ĢŒų‰Ź‚ĶŲ–¾‚³‚ź‚Ä‚¢‚Č‚¢B

y‰š ąz

‰”­APL ‚ĢŠ°‰š“±“ü—Ć–@‚É‚Ø‚¢‚Ä‚ĶƒI[ƒ‹ƒgƒ‰ƒ“ƒXŒ^ƒŒƒ`ƒmƒCƒ“Ž_iATRAj‚Ģ“±“ü‚É‚ę‚čŒø­‚µ‚½‚Ę‚ĶŒ¾‚¦‘ŸŠķoŒŒ‚ŖÅ‚ą‘å‚«‚Č”ńŠ°‰š‚ĢŒ“ˆö‚Å‚ ‚éBJapan Adult Leukemia Study GroupiJALSGjAPL97 Œ¤‹†‚Å‚Ķ283 —į’†‚Ģ”ńŠ°‰š16 —į‚Ģ‚¤‚æ9 —įi3“j‚É”]oŒŒ‚ā”xoŒŒ‚É‚ę‚鑁ŠśŽ€–S‚š”F‚ß‚½1jBPETHEMA Œ¤‹†‚Å‚ąoŒŒ‚É‚ę‚鑁ŠśŽ€–S‚š5“‚É”F‚ß‚Ä‚¢‚é2jB‚³‚ē‚ɁCƒXƒEƒF[ƒfƒ“‚ĢlŒū“®‘Ō’²ø‚Å‚Ķ105 —į‚ĢAPL —į‚Ģ29“‚Ŗ‘ŠśŽ€–S‚Å‚ ‚čC‚¤‚æ41“‚ɏoŒŒ‚š”F‚ß‚½B‘ŠśŽ€–S‚Ģ35“‚ĶŽå‚ɐf’fˆČ‘O‚ɏoŒŒ‚š•¹”­‚µCATRA Ž”—Ć‚šŽó‚Æ‚Ä‚¢‚Č‚©‚Į‚½3jB‚±‚Ģ‚ꂤ‚ɁC—Տ°ŽŽŒ±‚É“o˜^‚³‚ź‚Č‚¢ŽĄ’nf—Ć—į‚Å‚ĶˆĖ‘R‘ŠśŽ€–S—į‚Ķ‘½‚¢‚Ę—\‘z‚³‚źC‘Šśf’f‚É‚ę‚éoŒŒ—\–h‚Ŗd—v‚Å‚ ‚éB

JALSG APL97 Œ¤‹†‚É‚Ø‚¢‚Ä‘ŸŠķoŒŒ‚š•¹”­‚µ‚½18 —į‚Ę•¹”­‚µ‚Č‚©‚Į‚½261 —į‚š”äŠr‚µCoŒŒ‚Ģ‚ƒŠƒXƒNˆöŽq‚Ķ‘½•Ļ—Ź‰šĶ‚É‚Ø‚¢‚Ä’įƒtƒBƒuƒŠƒmƒQƒ“ŒŒĒiƒ100 mg/dLjC”’ŒŒ‹…”‚’li„20,000/ƒŹLj‚Ø‚ę‚Ń‘Sgó‘Ōiperformance statusFPSj2`3 ‚Å‚ ‚Į‚½1jB

APL ‚Ģ‹ĆŒÅˆŁķ‚Ķü—n‰ßč‚ŖŽå‘Ģ‚Å‚ ‚čCŒŒ¬”Ā—AŒŒ‚É‚ę‚čŒŒ¬”Ā”‚š‚Å‚«‚ź‚Ī50,000/ƒŹL ˆČćC­‚Č‚­‚Ę‚ą30,000/ƒŹL ˆČćC“€Œ‹ŒŒŸ÷‚É‚ę‚čƒtƒBƒuƒŠƒmƒQƒ“150 mg/dL ˆČć‚š–Ś•W‚Ę‚·‚é•ā[—Ć–@‚Ŗ„§‚³‚ź‚éBATRA —Ć–@‚É‚ę‚銰‰š“±“üŽž‚Ģ‚±‚ź‚ē‚Ģ–Ś•W’l‚š”äŠr‚µ‚½ŽŽŒ±‚Ķ‚Č‚¢B‰»Šw—Ć–@‚Ģ‚Ż‚ĢŽž‘ć‚ɁCŒŒ¬”Ā‚Ø‚ę‚ŃƒtƒBƒuƒŠƒmƒQƒ“‚Ģ•ā[—Ć–@‚Ģ‚ŻCƒwƒpƒŠƒ“‚É‚ę‚éR‹ĆŒÅ—Ć–@‚Ø‚ę‚Ńƒgƒ‰ƒlƒLƒTƒ€Ž_“™‚É‚ę‚éRü—n—Ć–@‚Ģ3 ŒQ‚É‚ę‚éŒć•ūŽ‹“I‰šĶ‚Ŗs‚ķ‚źCŠ°‰š—¦CoŒŒ‚É‚ę‚鑁ŠśŽ€–S‚É—LˆÓ·‚š”F‚ß‚Č‚©‚Į‚½4jB’į•ŖŽqƒwƒpƒŠƒ“Cƒ_ƒiƒoƒƒCƒhƒiƒgƒŠƒEƒ€Cˆā“`Žq‘g‚ŻŠ·‚¦ƒgƒƒ“ƒ{ƒ‚ƒWƒ…ƒŠƒ“‚Č‚Ē‚É‚ę‚éR‹ĆŒÅ—Ć–@‚Ø‚ę‚Ńƒgƒ‰ƒlƒLƒTƒ€Ž_‚É‚ę‚éRü—n—Ć–@‚Ģ—LŒų«‚Ķ\•ŖŲ–¾‚³‚ź‚Ä‚¢‚Č‚¢Bƒgƒ‰ƒlƒLƒTƒ€Ž_‚É‚ę‚éRü—n—Ć–@‚É‚Ā‚¢‚Ä‚ĶATRA ‚Ę•¹—p‚·‚邱‚Ę‚ÅŒŒšĒ‚Ŗ‘‰Į‚·‚éŠėŒÆ‚Ŗ‚ ‚é5jB’į•ŖŽqƒwƒpƒŠƒ“Cƒ_ƒiƒoƒƒCƒhƒiƒgƒŠƒEƒ€CƒƒVƒ‹Ž_ƒKƒxƒLƒT[ƒg‚āƒƒVƒ‹Ž_ƒiƒtƒ@ƒ‚ƒXƒ^ƒbƒg‚Ģ‡¬’`”’•Ŗ‰šy‘f‘jŠQÜ‚É‚ę‚éR‹ĆŒÅ—Ć–@‚É‚Ā‚¢‚Ä‚ĶC”äŠrŽŽŒ±‚Ģ•ń‚É–R‚µ‚¢Bˆā“`Žq‘gŠ·‚¦ƒgƒƒ“ƒ{ƒ‚ƒWƒ…ƒŠƒ“Žg—p‚É‚Ā‚¢‚Ä‚ĶCDIC ‚©‚ē‚Ģ‘Šś—£’E‚ʐV‘N“€Œ‹ŒŒŸ÷—AŒŒ—Ź‚ĢŒø­‚Ŗ“¾‚ē‚ź‚½‚Ę‚Ģ•ń‚Ŗ‚ ‚é‚ŖC­”—į‚Ģ‰šĶ‚Å‚ ‚é6jBAPL ‚Ģ‹ĆŒÅˆŁķ‚ĶĒ—į‚É‚ę‚Į‚Ä‘å‚«‚­ˆŁ‚Č‚čC“Į’č‚ĢĒ—į‚É‚ĶR‹ĆŒÅ—Ć–@‚āRü—n—Ć–@‚Ŗ—LŒų‚ȉĀ”\«‚ą‚ ‚čC”Œć‚ĢŒ¤‹†‰Ū‘č‚Å‚ ‚éB‚³‚ē‚ɁCATRA ‚ĶAPL ‚Ģ‹ĆŒÅˆŁķ‚š’¼Ś‚Ø‚ę‚ŃŠŌŚ‚ɉü‘P‚·‚é‚Ģ‚ŁC—Տ°“I‚ÉAPL ‚Ŗ‹^‚ķ‚ź‚½ź‡‚É‚ĶPML-RARA ‚ĢŒ‹‰Ź‚š‘Ņ‚½‚ø‚É‘Šś‚ÉATRA ‚šŠÜ‚ŽŽ”—Ć‚šŠJŽn‚·‚邱‚Ę‚ĶoŒŒ—\–h‚É‚Ā‚Č‚Ŗ‚é7jB

yŽQl•¶Œ£z

1j Yanada M, et al. Severe hemorrhagic complications during remission induction therapy for acute promyelocytic leukemia : incidence, risk factors, and influence on outcome. Eur J Haematol. 2007 ; 78i3j : 213-9.i3iDiij

2j de la Serna J, et al. Causes and prognostic factors of remission induction failure in patients with acute promyelocytic leukemia treated with all-trans retinoic acid and idarubicin. Blood. 2008 ; 111 i7j : 3395-402.i3iAj

3j Lehmann S, et al. Continuing high early death rate in acute promyelocytic leukemia : a population-based report from the Swedish Adult Acute Leukemia Registry. Leukemia. 2011 ; 25i7j : 1128-34.i3iDj

4j Rodeghiero F, et al. Early deaths and anti-hemorrhagic treatments in acute promyelocytic leukemia. A GIMEMA retrospective study in 268 consecutive patients. Blood. 1990 ; 75i11j : 2112-7.i3iiiBj

5j Brown JE et al. All-trans retinoic acid iATRAj and tranexamic acid : a potentially fatal combination in acute promyelocytic leukaemia. Br J Haematol. 2000 ; 110i4j : 1010-2.i3iiAj

6j Ikezoe T, et al. Recombinant human soluble thrombomodulin safely and effectively rescues acute promyelocytic leukemia patients from disseminated intravascular coagulation. Leukemia Res. 2012 ; 36i11j : 1398-402.i3iiDj

7j Di Bona E, et al. Early haemorrhagic morbidity and mortality during remission induction with or without all-trans retinoic acid in acute promyelocytic leukaemia. Br J Haematol. 2000 ; 108i4j : 689-95.i2Aj


CQ4

APL •Ŗ‰»ĒŒóŒQ‚ĢŽ”—Ć‚Ķ‰½‚ŖŠ©‚ß‚ē‚ź‚é‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2A
APL •Ŗ‰»ĒŒóŒQiDSj‚Ģf’f‚ĘŽ”—Ć‚Ģƒ|ƒCƒ“ƒg‚Ķ—Տ°Ēó‚É‚ę‚鑁Šś”­Œ©‚ʁCDS ‚ĢdĒ“x‚É‚ę‚éATRA ‚āATO “Š—^‚Ģ’†Ž~C•›t”ēŽæƒXƒeƒƒCƒh‚Ģ‘ŠśŠJŽn‚Ŗ„§‚³‚ź‚éB‰fŽž”’ŒŒ‹…”‚Ŗ‘½‚¢Ē—įCŽ”—Ć’†‚É”’ŒŒ‹…‚Ŗ‘‰Į‚·‚éĒ—į‚Å‚ĶC‰»Šw—Ć–@‚Ģ•¹—p‚Ŗ„§‚³‚ź‚éBƒvƒŒƒhƒjƒ]ƒƒ“‚Ģ—\–h“Š—^‚ĶŠ©‚ß‚ē‚ź‚Č‚¢B

y‰š ąz

1DĒŒó‚ʐf’f

ƒtƒ‰ƒ“ƒX‚ĢAPL93 Œ¤‹†1jC•Ä‘‚ĢIntergroup study 0129 Œ¤‹†2jC‚Ø‚ę‚ŃƒXƒyƒCƒ“‚ĢLPA96 ‚ĘLPA99 Œ¤‹†3j‚É‚ę‚ź‚ĪCAPL •Ŗ‰»ĒŒóŒQidifferentiation syndromeFDSj‚Ģ”­¶—¦‚Ķ2.5`26“‚Å‚ ‚čC‘SĒ—į‚É‘Ī‚·‚鎀–S—¦‚Ķ0`3.4“‚Å‚ ‚éBDS ‚Ģ”­Œ»“śif’f“śj‚ĶCŽ”—ĆŠJŽn‚©‚ē’†‰›’l7`11 “śi0`47 “śj‚Å‚ ‚éB•p“x‚Ģ‚‚¢ĒŒó‚ĶC①•pŒÄ‹zCŒÄ‹z¢“ļC’įŽ_‘fŒŒĒiSpO2 ‚Ģ’į‰ŗjC②•s–¾”MC③‘Ģd‘‰ĮC④•‚ŽīC⑤ŒŒˆ³’į‰ŗC⑥‹}«t•s‘SC‚¤‚ĮŒŒ«S•s‘SC⑦”xZ‰eC‹¹…CS”X…i‹¹•”X üCCT ‚Č‚ĒjC‚Å‚ ‚éB

2DDS ”­Ē‚ĢŠėŒÆˆöŽq

Š°‰š“±“ü—Ć–@‚Å‚Ķ‰fŽž”’ŒŒ‹…”‚Ģ­‚Č‚¢Ē—į‚ŃI[ƒ‹ƒgƒ‰ƒ“ƒXŒ^ƒŒƒ`ƒmƒCƒ“Ž_iATRAj’P“ĘŽ”—Ć’†‚Ģź‡C”’ŒŒ‹…‘‰Į‚Ŗ‚Ż‚ē‚ź‚ź‚Ī‘¬‚ā‚©‚ɉ»Šw—Ć–@‚š’Ē‰Į‚·‚éB‰fŽž‚Ģ”’ŒŒ‹…”‚ĘDS ‚Ģ”­Ē—¦‚Ķ•K‚ø‚µ‚ą‘ŠŠÖ‚µ‚Č‚¢‚±‚Ę‚Ŗ’m‚ē‚ź‚Ä‚¢‚é‚ŖCdĒ‚ĢDS ‚Ķ”’ŒŒ‹…‘‰ĮŽžC“Į‚É5,000/ƒŹL ˆČć‚Ŗ—LˆÓ‚Č”­ĒŠėŒÆˆöŽq‚Ę‚µ‚ÄŽw“E‚³‚ź‚Ä‚¢‚é1j`3jB

3DŽ”—Ć

DS ‚ĢdĒ“x‚É‚ę‚čATRA ‚āˆŸƒqŽ_iATOj‚š‹x–ņ‚·‚邱‚ʂʁCƒfƒLƒTƒƒ^ƒ]ƒ“iDEXj‚Ü‚½‚Ķƒƒ`ƒ‹ƒvƒŒƒhƒjƒ]ƒƒ“ƒpƒ‹ƒX—Ć–@‚Å‚ ‚éBDS ‚ŖdĒ‰»‚µ‚½ź‡it•s‘S‚āŒÄ‹z•s‘S‚É‚ę‚éW’†Ž”—Ć‚š—v‚·‚éj‚āDEX ‚ĢŒų‰Ź‚Ŗ‚Ż‚ē‚ź‚Č‚¢ź‡‚É‚Ģ‚Ż‹x–ņ‚·‚é‚Ę‚·‚éˆÓŒ©‚ą‚ ‚éB‚µ‚©‚µCDS ‚Ģ‘½–Ź“I‚ȏĒŒó‚ÉŠÖ‚·‚é–¾Šm‚ȏdĒ“x‹K€‚Ŗ‚Č‚¢‚½‚߁CDS f’fŽž‚Å‚ĢATRA ‚ĢŽ”—ĆŒų‰Ź‚ĘDS ‚É‚ę‚é‘ŸŠķ•s‘S‚ĢdĒ“x‚šĒ—į‚²‚Ę‚Él—¶‚µ‚Ä”»’f‚·‚éBATRA ‚ĶĒó‚ŖŠ®‘S‚ɏĮŽø‚µ‚Ä‚©‚ēÄŠJ‚·‚é‚ŖCÄŠJŽž‚Ģ“Š—^—Ź‚Ķ‰‰ń‚Ģ75“—Ź‚©‚ēŠJŽn‚µC3`5 “śŠŌĒó‚ŖÄ”R‚µ‚Č‚¢‚±‚Ę‚šŠm”F‚µ‚Ä‚©‚ēŒ³‚Ģ—Ź‚É–ß‚µ‚Ä‚¢‚­•ū–@‚ą‚ ‚éBDEX ‚Ķ10 mg ‚š1 “ś2 ‰ńŒoĆ–¬“I‚É“Š—^‚·‚éB“Š—^ŠJŽn‚ĶĒó‚āĒŒó‚ŖoŒ»‚µ‚½‘Šś‚Å‚ ‚é‚Ł‚Ē–]‚Ü‚µ‚­CĒó‚ŖŠ®‘S‚ɏĮŽø‚·‚é‚Ü‚ÅŒp‘±‚·‚éB

4D”­Ē—\–h‚Ę‚µ‚Ä‚Ģ‰»Šw—Ć–@‚Ģ•¹—pC’Ē‰Į

‰»Šw—Ć–@‚Ģ•¹—p‚É‚ę‚č”’ŒŒ‹…”‚Ģ‘‰Į‚Ŗ—}§‚³‚źCDS ‚Ģ”­Ē‚š‚Ø‚³‚¦‚Ä‚¢‚é‚ʍl‚¦‚ē‚ź‚éBƒtƒ‰ƒ“ƒX‚ĢAPL93 Œ¤‹†‚Å‚Ķ4jC”’ŒŒ‹…5,000/ƒŹL –¢–ž‚ĢĒ—į‚ŁCATRA ‚ʉ»Šw—Ć–@“ÆŽžiŽĄŪ‚É‚Ķ3 “śŒćjŠJŽnŒQ‚Ę’P“ĘATRA Ž”—ĆŒQ‚š”äŠr‚µ‚Ä‚¢‚é‚ŖC‘OŽŅ‚ÅDS ”­Ē—¦‚Ķ’į‚©‚Į‚½B‚Ü‚½ATRA ’P“ĘŽ”—Ć‚Ģź‡Cday5 ‚Å6,000/ƒŹL ˆČćCday10 ‚Å10,000/ƒŹL ˆČćCday15 ‚Å15,000/ƒŹL ˆČć‚Ę‚Č‚ź‚Ī‘¬‚ā‚©‚ɉ»Šw—Ć–@‚Ŗ’Ē‰Į‚³‚ź‚é•ūj‚ŖŽ¦‚³‚ź‚Ä‚¢‚éB

5D•›t”ēŽæƒXƒeƒƒCƒh‚Ģ—\–h“Š—^

•›t”ēŽæƒXƒeƒƒCƒh‚Ģ—\–h“I“Š—^‚É‚ę‚čDS ‚ĢŽ€–S—¦‚šŒø­‚³‚¹‚½‚Ę‚·‚éƒGƒrƒfƒ“ƒX‚Ķ–R‚µ‚­C•W€Ž”—Ć‚É‚Ķ‚Č‚Į‚Ä‚¢‚Č‚¢BƒXƒyƒCƒ“‚Å‚ĢLPA99 Œ¤‹†‚Å‚ĶCƒvƒŒƒhƒjƒ]ƒƒ“0.5 mg/kg/“ś‚šday1`15 ‚Ü‚Å“ą•ž‚µDS ‚Ģ”­Ē—\–hŒų‰Ź‚šŒŸ“¢‚µ‚½‚ŖC‰ß‹Ž‚ĢLPA96 Œ¤‹†‚Ę”äŠr‚µ‚Ä”­Ē—¦‚ɍ·‚Ķ‚Č‚©‚Į‚½5jB

yŽQl•¶Œ£z

1j De Botton S, et al. Incidence, clinical features, and outcome of All trans-retinoic acid syndrome in 413 cases of newly diagnosed acute promyelocytic leukemia. Blood. 1998 ; 92i8j : 2712-8.i3iiiDivj

2j Tallman MS, et al. Clinical description of 44 patients with acute promyelocytic leukemia who developed the retinoic acid syndrome. Blood. 2000 ; 95i1j : 90-5.i3iiiDivj

3j Montesinos P, et al. Differentiation syndrome in patients with acute promyelocytic leukemia treated with all-trans retinoic acid and anthracycline chemotherapy : characteristics, outcome, and prognostic factors. Blood. 2009 ; 113i4j : 775-83.i3iiiDivj

4j de Botton S, et al. Early onset of chemotherapy can reduce the incidence of ATRA syndrome in newly diagnosed acute promyelocytic leukemia iAPLj with low white blood cell counts : results from APL 93 trial. Leukemia. 2003 ; 17i2j : 339-42.i3iiiDivj

5j Sanz MA, et al. Risk-adapted treatment of acute promyelocytic leukemia with all-trans-retinoic acid and anthracycline monochemotherapy : a multicenter study by the PETHEMA group. Blood. 2004 ; 103i4j : 1237-43.i3iiiDivj


CQ5

–¢Ž”—ĆAPL ‚ĢATRA ‚ʉ»Šw—Ć–@‚É‚ę‚銰‰šŒć‚ĢŽŠ“K‚Č’nŒÅ‚ß—Ć–@‚Ķ‰½‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[1
Š°‰š“±“ü—Ć–@ŒćCŒŒ‰tŠw“IŠ®‘SŠ°‰š‚Ŗ’B¬‚³‚ź‚½ź‡Cˆų‚«‘±‚«ƒAƒ“ƒgƒ‰ƒTƒCƒNƒŠƒ“Œn–ņÜ‚Ü‚½‚ĶƒVƒ^ƒ‰ƒrƒ“•¹—p‚É‚ę‚é’nŒÅ‚ß—Ć–@‚š2 ‚Č‚¢‚µ3 ƒR[ƒXs‚¤‚±‚Ę‚Ŗ„§‚³‚ź‚éB

„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2B
Ž”—Ć‘O”’ŒŒ‹…”‚’l‚Ģ‚ƒŠƒXƒNŒQ‚Å‚ĶƒAƒ“ƒgƒ‰ƒTƒCƒNƒŠƒ“Œn–ņÜ‚ĘƒVƒ^ƒ‰ƒrƒ“•¹—p‚Ģ’nŒÅ‚ß—Ć–@‚Ŗ„§‚³‚ź‚éB

„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2A
’nŒÅ‚ß—Ć–@I—¹Žž‚Ģ•ŖŽq¶•ØŠw“IŠ°‰š‚Ŗ’·Šś¶‘¶‚Ģ•K{šŒ‚Å‚ ‚čCœ‘PML-RARA ‚Ģ‘Ŗ’č‚Ŗ„§‚³‚ź‚éB

y‰š ąz

ƒAƒ“ƒgƒ‰ƒTƒCƒNƒŠƒ“Œn–ņÜmƒCƒ_ƒ‹ƒrƒVƒ“iIDRjCƒ_ƒEƒmƒ‹ƒrƒVƒ“iDNRjCƒ~ƒgƒLƒTƒ“ƒgƒƒ“iMITjn‚ŖŠī–{‚Å‚ ‚é‚ŖC‚±‚ź‚É•¹—p‚³‚ź‚éƒVƒ^ƒ‰ƒrƒ“iAraCj‚ĘƒI[ƒ‹ƒgƒ‰ƒ“ƒXŒ^ƒŒƒ`ƒmƒCƒ“Ž_iATRAj‚ĢˆÓ‹`‚ŖŒŸ“¢‚³‚ź‚Ä‚«‚½BAraC ‚š•¹—p‚µ‚Ä‚¢‚éƒtƒ‰ƒ“ƒX‚ĢAPL93 ‚Ø‚ę‚ŃAPL2000 Œ¤‹†‚ʁCAraC ‚š—p‚¢‚Č‚¢ƒXƒyƒCƒ“‚ĢLPA99 Œ¤‹†‚Ģ”äŠrŒŸ“¢‚Ŗs‚ķ‚ź‚½1jB’į‚Ø‚ę‚Ń’†ƒŠƒXƒNŒQ‚Å‚Ķ–³ƒCƒxƒ“ƒg¶‘¶Š„‡iEFSj‚ɍ·‚Ķ‚Č‚©‚Į‚½‚ŖC‚ƒŠƒXƒNŒQ‚Å‚ĶAPL93C2000 ŒQ‚Å82.2“CLPA99 ŒQ‚Å67.3“C‚Ę—LˆÓ‚ÉAraC •¹—pŒQ‚Ŗ—D‚ź‚Ä‚¢‚½BƒXƒyƒCƒ“‚ĢLPA2005 Œ¤‹†‚Å‚ĶC‰ß‹Ž‚ĢLPA99 Œ¤‹†‚š‰ü’ł‚µ‚ƒŠƒXƒNŒQ‚ÅAraC ‚š’Ē‰Į‚µ‚½‚Ę‚±‚ėCLPA99 Œ¤‹†‚ɔ䂵—D‚ź‚½¬Ń‚Ŗ“¾‚ē‚ź‚½2jBƒCƒ^ƒŠƒA‚ĢAIDA2000 Œ¤‹†‚Å‚Ķ‰ß‹Ž‚ĢAIDA0493 Œ¤‹†‚š‰ü’ł‚µC‘S—į‚ÅATRA ‚Ģ•¹—p‚ʍ‚ƒŠƒXƒNŒQ‚Å‚ĢAraC ‚Ģ•¹—p‚šs‚Į‚½‚Ę‚±‚ėC‚ƒŠƒXƒNŒQ‚ōĔ­—¦‚Ŗ—LˆÓ‚ÉŒø­‚µ‚½3jB‚—īŽŅ‚Å‚ĶC’nŒÅ‚ß—Ć–@‚Å‚ĢŠ“õĒ‚Č‚Ē‚É‚ę‚鎔—ĆŠÖ˜AŽ€–SiTRMj‚Ŗ‘½‚¢‚½‚߁C‚ę‚č“Ő«‚Ģ­‚Č‚¢‚ą‚Ģ‚Ŗ„§‚³‚ź‚é4jBÅ‚ąd—v‚Čƒ|ƒCƒ“ƒg‚ĶC’nŒÅ‚ß—Ć–@I—¹Žž‚É•ŖŽq¶•ØŠw“IŠ°‰š‚Ŗ’B¬‚³‚ź‚Ä‚¢‚邱‚Ę‚Å‚ ‚éB‚±‚ĢŽž“_‚ÅRQ-PCR –@‚É‚ę‚鍜‘×–E‚ĢPML-RARA ‚Ŗ—z«‚Ģź‡‚Ķ—\Œć•s—Ē‚Å‚ ‚邽‚߁CˆŸƒqŽ_iATOjŽ”—Ć‚Č‚Ē‚Ģ“ńŽŸŽ”—Ć‚šs‚¢CPML-RARA ‚Ŗ‰A«‚É‚Č‚ź‚ĪŽ©‰Ę‘¢ŒŒŠ²×–EˆŚA‚Ŗl—¶‚³‚ź‚éB“ńŽŸŽ”—ĆŒć‚ąPML-RARA ‚Ŗ—z«‚Ģź‡‚ĶC“ÆŽķ‘¢ŒŒŠ²×–EˆŚA‚Ŗl—¶‚³‚ź‚é5jB‘¼‚ĢAML ‚Ģ‚ꂤ‚ÉŠ°‰šŒć‚É’†•_ŒoŒnÄ”­—\–h‚Ģ‚½‚߃ƒgƒgƒŒƒLƒT[ƒgiMTXjCAraC ‚Ģ‘’‚šs‚¤‚±‚Ę‚ą‚ ‚é‚ŖC‚ƒŠƒXƒNŒQˆČŠO‚Å‚Ķ“Į‚ɐ„§‚³‚ź‚Ä‚¢‚Č‚¢6jB

yŽQl•¶Œ£z

1j Adés L, et al. Treatment of newly diagnosed acute promyelocytic leukemia iAPLj : a comparison of French-Belgian-Swiss and PETHEMA results. Blood. 2008 ; 111i3j : 1078-84.i3iiiDij

2j Sanz MA, et al. Risk-adapted treatment of acute promyelocytic leukemia based on all-trans retinoic acid and anthracycline with addition of cytarabine in consolidation therapy for high-risk patients : further improvements in treatment outcome. Blood. 2010 ; 115i25j : 5137-46.i3iiiDiij

3j Lo-Coco F, et al. Front-line treatment of acute promyelocytic leukemia with AIDA induction followed by risk-adapted consolidation for adults younger than 61 years : results of the AIDA-2000 trial of the GIMEMA Group. Blood. 2010 ; 116i17j : 3171-9.i3iiiDiij

4j Ades L et al. Outcome of acute promyelocytic leukemia treated with all trans retinoic acid and chemotherapy in elderly patients : the European group experience. Leukemia. 2005 ; 19i2j : 230-3.i3iiiDij

5j Esteve J et al. Outcome of patients with acute promyelocytic leukemia failing to front-line treatment with all-trans retinoic acid and anthracycline-based chemotherapyiPETHEMAj protocols LPA96 and LPA99 : Benefit of an early intervention. Leukemia. 2007 ; 21i3j : 446-52.i3iiiA/2Aj

6j Montesinos P et al. Central nervous system involvement at first relapse in patients with acute promyelocytic leukemia treated with all-trans retinoic acid and anthracycline monochemotherapy without intrathecal prophylaxis. Haematologica. 2009 ; 94i9j : 1242-9.i3iiiDij


CQ6

–¢Ž”—ĆAPL ‚ĢŠ°‰š—į‚ɂ؂Ƃ鎊“K‚ČˆŪŽ—Ć–@‚Ķ‰½‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2A
’nŒÅ‚ß—Ć–@I—¹Žž‚ÉRT-PCR –@‚É‚ę‚éPML-RARA ‚Ŗ‰A«‰»‚µ‚Ä‚¢‚邱‚Ę‚ŖŠm”F‚³‚ź‚½ź‡C‚ƒŠƒXƒNŒQ‚Å‚ĶATRA “ą•ž‚š’†S‚Ę‚µ‚½ˆŪŽ—Ć–@‚Ŗl—¶‚³‚ź‚éB

y‰š ąz

ˆŪŽ—Ć–@‚ÉŠÖ‚µ‚Ä3 ‚Ā‚Ģd—v‚Č•ń‚Ŗ‚ ‚éBƒCƒ^ƒŠƒA‚ĢAIDA0493 Œ¤‹†‚Å‚ĶƒI[ƒ‹ƒgƒ‰ƒ“ƒXŒ^ƒŒƒ`ƒmƒCƒ“Ž_iATRAjCATRA/ƒƒgƒgƒŒƒLƒT[ƒgiMTXj/ƒƒ‹ƒJƒgƒvƒŠƒ“i6MPjCMTX/6MPCˆŪŽ—Ć–@‚Č‚µC‚Ģ4 ŒQ‚Ģ”äŠrŒ¤‹†‚Ŗs‚ķ‚ź‚½‚ŖC12 ”N–³•a¶‘¶Š„‡iDFSj‚Ķ4 ŒQ‚É—LˆÓ·‚Ķ‚Č‚©‚Į‚½1jBƒtƒ‰ƒ“ƒX‚ĢAPL93 Œ¤‹†‚Å‚Ķ“Æ‚¶4 ŒQ‚Å‚Ģ”äŠrŒŸ“¢‚šs‚Į‚Ä‚¢‚é‚ŖC10 ”N—ŻĻÄ”­—¦‚ĶATRA/MTX/6MP ŒQ‚ŖÅ‚ą’į‚­C“Į‚ɏ‰fŽž”’ŒŒ‹…‚’lŒQiWBC„5,000/ƒŹLj‚É‚Ø‚¢‚Ä—LŒų‚Å‚ ‚Į‚½2jB‚Ü‚½ˆŪŽ—Ć–@‚Č‚µ‚É”ä‚ׂāCATRA ’P“ʁCMTX/6MP ‚ą—LŒų‚Å‚ ‚Į‚½B‚ķ‚Ŗ‘‚ĢAPL97 Œ¤‹†‚Å‚Ķ3jC6 ƒR[ƒX‚ĢŽå‚É“_“HĆ’‚É‚ę‚鑽Ü•¹—p‰»Šw—Ć–@‚Ę–³Ž”—ĆŠĻŽ@ŒQ‚Ģ‘O•ūŒü“I”äŠrŒ¤‹†‚ŖŽĄŽ{‚³‚ź‚½‚ŖC—¼ŒQ‚É—LˆÓ·‚Ķ‚Č‚­C‚Ž‚µ‚ė‰»Šw—Ć–@ŒQ‚Å•s—Ē‚ČŒXŒü‚É‚ ‚Į‚½B‚µ‚½‚Ŗ‚Į‚Ä‘½Ü•¹—p‰»Šw—Ć–@‚ĶˆŪŽ—Ć–@‚Ę‚µ‚Ä—LŒų‚Å‚Č‚­CŠ©‚ß‚ē‚ź‚Č‚¢BˆČć‚ę‚čC’įƒŠƒXƒNŒQi”’ŒŒ‹……10,000/ƒŹLCŒŒ¬”Ā„40,000/ƒŹLjC‚Ø‚ę‚Ń’†ƒŠƒXƒNŒQi”’ŒŒ‹……10,000/ƒŹLCŒŒ¬”Ā…40,000/ƒŹLj‚Å‚ĢÅ“K‚ČˆŪŽ—Ć–@‚Ķ”Œć‚Ģ‰Ū‘č‚Å‚ ‚é‚ŖC‚ƒŠƒXƒNŒQi”’ŒŒ‹…„10,000/ƒŹLj‚Å‚ĶATRA “ą•ž‚š’†S‚Ę‚µ‚½ˆŪŽ—Ć–@‚Ŗl—¶‚³‚ź‚é‚Å‚ ‚ė‚¤B‚½‚¾‚µC–{“K‰ž‚Å‚Ģ“Š—^‚Ķ‘“ą“K‰žŠO‚Å‚ ‚éB

yŽQl•¶Œ£z

1j Avvisati G, et al. AIDA 0493 protocol for newly diagnosed acute promyelocytic leukemia : very long-term results and role of maintenance. Blood. 2011 ; 117i18j : 4716-25.i1iiDiij

2j Ades L, et al. Very long-term outcome of acute promyelocytic leukemia after treatment with all-trans retinoic acid and chemotherapy : the European APL Group experience. Blood. 2010 ; 115 i9j : 1690-6.i1iiDij

3j Asou N, et al. A randomized study with or without intensifi ed maintenance chemotherapy in patients with acute promyelocytic leukemia who have become negative for PML-RARalpha transcript after consolidation therapy : The Japan Adult Leukemia Study GroupiJALSGj APL97 study. Blood. 2007 ; 110i1j : 59-66.i1iiDiij


CQ7

Ä”­APL ‚ĢŽŠ“K‚ȍĩ°‰š“±“ü—Ć–@‚Ķ‰½‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2A
RQ-PCR –@‚É‚ę‚鍜‘×–E‚ĢPML-RARA ‚Ŗ—z«‰»‚·‚ź‚ĪCŒŒ‰tŠw“IŠ°‰š‚Å‚ ‚Į‚Ä‚ąÄŠ°‰š“±“ü—Ć–@‚šŠJŽn‚·‚éB

„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2A
ˆŸƒqŽ_‚šŠÜ‚ŽƒŒƒWƒƒ“‚Ŗ‘ęˆź‘I‘š‚Ę‚Č‚éB

„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2B
ˆŸƒqŽ_‚ŖŽg—p‚Å‚«‚Č‚¢‚Ę‚«CƒQƒ€ƒcƒdƒ}ƒu ƒIƒ]ƒKƒ}ƒCƒVƒ“‚Ü‚½‚Ķƒ^ƒ~ƒoƒƒeƒ“‚šŠÜ‚ŽƒŒƒWƒƒ“‚ĢŽg—p‚šl—¶‚·‚éB

y‰š ąz

Š°‰šŒć‚ĢRQ-PCR –@‚É‚ę‚éPML-RARA ‚Ģƒ‚ƒjƒ^ƒŠƒ“ƒOŽž‚ɁCŒŒ‰tŠw“I‚ÉŠ°‰šó‘Ō‚Å‚ ‚č‚Č‚Ŗ‚ēCœ‘ŒŸø‚ÅPML-RARA ‚Ŗ—z«‰»‚µ‚Ä‚¢‚éĒ—į‚Å‚ĶC‚Ł‚Ę‚ń‚Ē‚ĢĒ—į‚ŖŒć‚ÉŒŒ‰tŠw“IÄ”­‚š‚«‚½‚·1j‚±‚Ę‚©‚ēC•ŖŽq¶•ØŠw“IÄ”­‚Ģó‘Ō‚ÅŽ”—Ć‚šŽn‚ß‚½•ū‚ŖC‚»‚ĢŒć‚ĢÄ”­—¦E‘S¶‘¶Š„‡iOSj‚Ę‚ą‚É—LˆÓ‚É—D‚ź‚é2j3jBƒI[ƒ‹ƒgƒ‰ƒ“ƒXŒ^ƒŒƒ`ƒmƒCƒ“Ž_iATRAj‚šƒx[ƒX‚Ę‚µ‚½Š°‰š“±“ü—Ć–@Ž{sĒ—į‚ĢÄ”­‚Å‚ĶCˆŸƒqŽ_iATOj‚Ģ“Š—^‚É‚ę‚č80`90“‚ĢĒ—į‚Å•ŖŽq¶•ØŠw“IÄŠ°‰š‚Ŗ“¾‚ē‚źC1`3 ”N‚ĢOS ‚Ķ50`70“‚É’B‚·‚é4j`8jB

ATO ’P“Ę“Š—^Žž‚É‚Ķ”’ŒŒ‹…”‘‰Į‚š”¼”‚É”F‚߁C25“‚ŖAPL •Ŗ‰»ĒŒóŒQidifferentiation syndromeFDSj‚š”­Ē‚·‚é5j‚±‚Ę‚©‚ēC”’ŒŒ‹…‘‰Į—į‚Å‚ĶƒAƒ“ƒgƒ‰ƒTƒCƒNƒŠƒ“Œn‚Č‚Ē‚ĢR‚Ŗ‚ńÜ‚š•¹—p‚µ‚½‚Ł‚¤‚ŖˆĄ‘S‚Å‚ ‚éBDS ”­ĒŽž‚ĶC•›t”ēŽæƒzƒ‹ƒ‚ƒ“‚Ģ“Š—^‚šŠJŽn‚µCdĒ‚Å‚ ‚ź‚ĪATO ‚Ģ“Š—^‚š’†Ž~‚·‚éBATO ‚ĶCQT ‰„’·‚Ģ•›ģ—p‚Ŗ‚ ‚čC’v–½“I‚Čtorsade de pointes ƒ^ƒCƒv‚ĢSŽŗ«•s®–¬‚šˆų‚«‹N‚±‚·‚±‚Ę‚Ŗ‚ ‚éB‚»‚Ģ‚½‚߁CATO “Š—^ŠśŠŌ’†‚ĶS“d}‚š’芜“I‚Ƀ‚ƒjƒ^ƒŠƒ“ƒO‚µCŒŒ““d‰šŽæ”Z“x‚š“K³‚ɈŪŽ‚·‚éiK„4.0 mEq/dLCMg„1.8 mg/dLjB

ATO ‚ĘATRA ‚Ķin vitro ‚ŁCAPL ×–E‚É‘Ī‚µ‚Ä‘Šę“I‚ɍģ—p‚·‚邱‚Ę‚Ŗ’m‚ē‚ź‚Ä‚¢‚é‚ŖC¬‹K–Ķ‚Čƒ‰ƒ“ƒ_ƒ€‰»”äŠrŽŽŒ±‚É‚Ø‚¢‚āCATO ‚ÉATRA ‚š’Ē‰Į‚µ‚½ŒQ‚É—\Œć‚Ģ‰ü‘P‚Ŗ‚Ż‚ē‚ź‚Č‚©‚Į‚½‚±‚Ę‚©‚ēCATRA Ž”—ĆŒć‚ĢÄ”­—į‚Å‚ĶATO ‚ÉATRA ‚š•¹—p‚·‚éˆÓ‹`‚Ķ‘å‚«‚­‚Č‚¢‚ʍl‚¦‚ē‚ź‚Ä‚¢‚é8jB

ATO ‚šŽg—p‚Å‚«‚Č‚¢ź‡CƒQƒ€ƒcƒdƒ}ƒu ƒIƒ]ƒKƒ}ƒCƒVƒ“iGOj‚Ŗ‘ęˆź‘I‘š‚Ę‚Č‚éB•ŖŽqÄ”­—į‚É‘Ī‚·‚éGO ’PÜ‚Ģ“Š—^‚Å‚ĶC2 ‰ń“Š—^‚Å81.8“‚ĢĒ—į‚É•ŖŽq¶•ØŠw“IÄŠ°‰š‚Ŗ“¾‚ē‚ź‚½‚Ę•ń‚³‚ź‚Ä‚¢‚é9jBGO ‚Ķ—Ž““•ĀĒĒŒóŒQ‚š‚«‚½‚·‰Ā”\«‚Ŗ‚ ‚čCˆŚA—\’č—į‚É‚ĶŽg—p‚š”š‚Æ‚é‚©CˆŚA‚܂łɈź’芜ŠŌ‚š‚ ‚Æ‚é•K—v‚Ŗ‚ ‚éB‡¬ƒŒƒ`ƒmƒCƒ“Ž_ƒ^ƒ~ƒoƒƒeƒ“‚É‚Ā‚¢‚Ä‚ĶCATRA Ž”—ĆŒć‚ĢÄ”­—į‚É‘Ī‚·‚é‘ęⅡ‘ŠŽŽŒ±‚Å58“‚ĢÄŠ°‰š“±“ü—¦‚Å‚ ‚Į‚½‚Ŗ10jCŒ»Žž“_‚Å‚ĶATOCGO ‚ÉŽŸ‚®Ž”—Ć‚Ęl‚¦‚ē‚ź‚éB

yŽQl•¶Œ£z

1j Diverio D, et al. Early detection of relapse by prospective reverse transcriptase-polymerase chain reaction analysis of the PML/RARalpha fusion gene in patients with acute promyelocytic leukemia enrolled in the GIMEMA-AIEOP multicenterg AIDAh trial. Blood. 1998 ; 92i3j : 784-9.i1iiDivj

2j Lo Coco F, et al. Therapy of molecular relapse in acute promyelocytic leukemia. Blood. 1999 ; 94 i7j : 2225-9.i2Aj

3j Esteve J, et al. Outcome of patients with acute promyelocytic leukemia failing to front-line treatment with all-trans retinoic acid and anthracycline-based chemotherapy iPETHEMA protocols LPA96 and LPA99j : benefit of an early intervention. Leukemia. 2007 ; 21i3j : 446-52.i3iiiAj

4j Soignet SL, et al. Complete remission after treatment of acute promyelocytic leukemia with arsenic trioxide. N Engl J Med. 1998 ; 339i19j : 1341-8.i3iiiDivj

5j Soignet SL, et al. United States multicenter study of arsenic trioxide in relapsed acute promyelocytic leukemia. J Clin Oncol. 2001 ; 19i18j : 3852-60.i3iiiAj

6j Niu C, et al. Studies on treatment of acute promyelocytic leukemia with arsenic trioxide : remission induction, follow-up, and molecular monitoring in 11 newly diagnosed and 47 relapsed acute promyelocytic leukemia patients. Blood. 1999 ; 94i10j : 3315-24.i3iiiDiij

7j Shigeno K, et al. Arsenic trioxide therapy in relapsed or refractory Japanese patients with acute promyelocytic leukemia : updated outcomes of the phase Ⅱ study and postremission therapies. Int J Hematol. 2005 ; 82i3j : 224-9.i3iiiAj

8j Raffoux E, et al. Combined treatment with arsenic trioxide and all-trans retinoic acid in patients with relapsed acute promyelocytic leukemia. J Clin Oncol. 2003 ; 21i12j : 2326-34.i1iiDivj

9j Lo-Coco F, et al. Gemtuzumab ozogamicin iMylotargj as a single agent for molecularly relapsed acute promyelocytic leukemia. Blood. 2004 ; 104i7j : 1995-9.i3iiiDivj

10j Tobita T, et al. Treatment with a new synthetic retinoid, Am80, of acute promyelocytic leukemia relapsed from complete remission induced by all-trans retinoic acid. Blood. 1997 ; 90i3j : 967-73.i3iiAj


CQ8

ˆŸƒqŽ_‚É‚ę‚éAPL ‘ę“ńŠ°‰š—į‚ĢŠ°‰šŒćŽ”—Ć‚Ę‚µ‚ĉ½‚ŖŠ©‚ß‚ē‚ź‚é‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2A
ˆŸƒqŽ_‚É‚ę‚éÄŠ°‰šAPL —į‚Å‚ĶˆŸƒqŽ_‚É‚ę‚é’nŒÅ‚ß—Ć–@ŒćCRQ-PCR–@‚É‚ę‚鍜‘PML-RARA ‰A«—į‚É‚ĶŽ©‰ĘˆŚA‚ŖŠ©‚ß‚ē‚ź‚éB

„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2A
œ‘PML-RARA —z«—į‚É‚Ķ“ÆŽķˆŚA‚ŖŠ©‚ß‚ē‚ź‚éB

„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2B
ˆŚA‚Ģ“K‰ž‚Ŗ‚Č‚¢Ē—į‚Å‚ĶƒQƒ€ƒcƒdƒ}ƒu ƒIƒ]ƒKƒ}ƒCƒVƒ“‚ŖŠ©‚ß‚ē‚ź‚éB

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ˆŸƒqŽ_iATOj‚Ģ“oźˆČ‘O‚É‚ĶƒI[ƒ‹ƒgƒ‰ƒ“ƒXŒ^ƒŒƒ`ƒmƒCƒ“Ž_iATRAj‚ʉ»Šw—Ć–@‚É‚ę‚éÄŠ°‰š“±“ü—Ć–@‚Ŗs‚ķ‚ź‚Ä‚¢‚½B‚»‚Ģ‘ę“ńŠ°‰šŠś‚ĢŽ©‰ĘˆŚA‚Ę“ÆŽķˆŚA‚š”äŠr‚µ‚½ƒˆ[ƒƒbƒpƒOƒ‹[ƒv‚ĢAPL91 ‚ĘAPL93 Œ¤‹†‚Å‚Ķ7 ”N‘S¶‘¶Š„‡iOSj‚ŖŽ©‰ĘˆŚAŒQ59.8“C“ÆŽķˆŚAŒQ51.8“‚ĘŽ©‰ĘˆŚA‚Ŗ—D‚ź‚Ä‚¢‚½1jB–³Ä”­¶‘¶Š„‡iRFSj‚Ķ79.4“ vs 92.3“‚Ę“ÆŽķˆŚA‚Ŗ—Ē‚©‚Į‚½‚ŖCŽ”—ĆŠÖ˜AŽ€–SiTRMj‚Ŗ6“‚Ę39“‚Ę“ÆŽķˆŚA‚É‘½‚©‚Į‚½BEuropean Group for Blood and Marrow TransplantationiEBMTj‚Ģ‘½”—į‚Ģ“o˜^—į‚š‰šĶ‚µ‚½Œ‹‰Ź‚Å‚ĶŽ©‰ĘˆŚA‚Ģ–³•a¶‘¶Š„‡iDFSj 51“C“ÆŽķˆŚA‚Å‚Ķ59“‚Å‚ ‚Į‚½2jB

ATO ‚É‚ę‚éÄŠ°‰šŒć‚ÉATO ƒx[ƒX‚ĢŠ°‰šŒćŽ”—Ć‚šs‚Į‚½ź‡C1.5 ”NOS 66“CRFS 56“‚Ęˆź’č‚Ģ’·Šś¶‘¶‚ą“¾‚ē‚ź‚é‚ŖCÄ”­‚ą”äŠr“I‘½‚¢3jBATO ‚É‚ę‚éÄŠ°‰šŒćCATO ‚Ģ‚Ż‚É‚ę‚é’nŒÅ‚ß—Ć–@‚ĘATO ‚ʉ»Šw—Ć–@‚Ģ•¹—p‚É‚ę‚é’nŒÅ‚ß—Ć–@‚ĢOS ‚š”äŠr‚µ‚½ćŠCƒOƒ‹[ƒv‚ĢŒ¤‹†‚Å‚ĶŒćŽŅ‚Ŗ—LˆÓ‚É—D‚ź‚Ä‚¢‚½4jBATO ‚É‚ę‚éÄŠ°‰šŒć‚ĢŽ©‰ĘˆŚA‚ĘATO ƒx[ƒX‚ĢŠ°‰šŒćŽ”—Ć‚š”äŠr‚µ‚½ƒCƒ“ƒh‚Ģ¬Ń‚Å‚ĶŽ©‰ĘˆŚAŒQ‚Ģ–³ƒCƒxƒ“ƒg¶‘¶Š„‡iEFSj 83“‚É‘Ī‚µ‚āCATO ŒQ‚Ķ34“‚Å‚ ‚Į‚½5jBATO ‚É‚ę‚éÄŠ°‰šŒć‚ɈŚA‚Ģ“K‰ž‚Ŗ‚Č‚¢Ē—į‚É‚ĶCATO Ž”—ĆŒć‚ĢÄ”­—į‚É‚ą—LŒų‚ČƒQƒ€ƒcƒdƒ}ƒu ƒIƒ]ƒKƒ}ƒCƒVƒ“iGOj‚Ŗ‘E‚ß‚ē‚ź‚é6jB

ÄŠ°‰šŒć‚ĢŽ”—Ć‚É‚Ā‚¢‚Ä‚Ķƒ‰ƒ“ƒ_ƒ€‰»”äŠrŽŽŒ±‚Ŗs‚ķ‚ź‚Ä‚¢‚Č‚¢‚Ģ‚ŏ\•Ŗ‚ČƒGƒrƒfƒ“ƒX‚Ķ‚Č‚¢B50 ĪˆČ‰ŗ‚ĢŒŒ‰ƒhƒi[‚Ŗ‚¢‚é—į‚É‚Ķ“ÆŽķˆŚA‚Ŗ‘I‘šŽˆ‚É‚Č‚é‚ŖC‚»‚źˆČŠO‚Å‚ĶŽ©‰ĘˆŚA‚Ŗ‘E‚ß‚ē‚ź‚éBŽ©‰ĘˆŚA‚ĶÄ”­—¦‚Ŗ”äŠr“I‚‚¢‚Ģ‚ōœ‘×–E‚ĢPML-RARA ‚Ŗ—z«‚ĢĒ—į‚É‚Ķ“ÆŽķˆŚAC‰A«—į‚É‚ĶŽ©‰ĘˆŚA‚Ŗ‚ę‚¢B

yŽQl•¶Œ£z

1j de Botton S, et al. Autologous and allogeneic stem-cell transplantation as salvage treatment of acute promyelocytic leukemia initially treated with all-trans-retinoic acid : a retrospective analysis of the European acute promyelocytic leukemia group. J Clin Oncol. 2005 ; 23i1j : 120-6.i2Aj

2j Sanz MA, et al. Hematopoietic stem cell transplantation for adults with acute promyelocytic leukemia in the ATRA era : a survey of the European Cooperative Group for Blood and Marrow Transplantation. Bone Marrow Transplant. 2007 ; 39i8j : 461-9.i3iiDij

3j Soignet SL, et al. United States multicenter study of arsenic trioxide in relapsed acute promyelocytic leukemia. J Clin Oncol. 2001 ; 19i18j : 3852-60.i3iiiAj

4j Niu C, et al. Studies on treatment of acute promyelocytic leukemia with arsenic trioxide : remission induction, follow-up, and molecular monitoring in 11 newly diagnosed and 47 relapsed acute promyelocytic leukemia patients. Blood. 1999 ; 94i10j : 3315-24.i3iiiDii/2Diij

5j Thirugnanam R, et al. Comparison of clinical outcomes of patients with relapsed acute promyelocytic leukemia induced with arsenic trioxide and consolidated with either an autologous stem cell transplant or an arsenic trioxide-based regimen. Biol Blood Marrow Transplant. 2009 ; 15i11j : 1479-84.i2Diij

6j Lo-Coco F, et al. Gemtuzumab ozogamicin iMylotargj as a single agent for molecularly relapsed acute promyelocytic leukemia. Blood. 2004 ; 104i7j : 1995-9.i3iiiDivj


CQ9

‚—īŽŅAPL ‚ĢŽŠ“K‚ČŽ”—Ć•ū–@‚Ķ‰½‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2A
‘Sgó‘Ō‚Ŗ”äŠr“I—ĒD‚ȍ‚—īŽŅ‚É‘Ī‚µ‚Ä‚ĶŽ”–ü‚š–ŚŽw‚µ‚½Ž”—Ć‚šŒv‰ę‚·‚é‚ŖCŽ”—Ć‹­“x‚ĶŽį”NŽŅ‚ę‚čŽć‚ß‚é‚ׂ«‚Å‚ ‚éB

„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2B
d“Ä‚Č•¹‘¶Ē‚šŽ‚æCƒAƒ“ƒgƒ‰ƒTƒCƒNƒŠƒ“ŒnR‚Ŗ‚ńÜ‚Ģ“Š—^‚Ŗ¢“ļ‚ȍ‚—īŽŅ‚É‘Ī‚µ‚Ä‚ĶCˆŸƒqŽ_‚šƒx[ƒX‚É‚µ‚½Ž”—Ć‚šs‚Į‚Ä‚ą‚ę‚¢B

y‰š ąz

‚—īŽŅAPL ‚ĶC‰”­Žž”’ŒŒ‹…”‚Ŗ­‚Č‚¢’įƒŠƒXƒN—į‚Ŗ‘½‚­CŽ”—Ć”½‰ž«‚ĶŽį”NŽŅ‚Ę•Ļ‚ķ‚ē‚Č‚¢‚ą‚Ģ‚ĢCŠ“õĒ‚Č‚Ē‚Ģ‡•¹Ē‚É‚ę‚Į‚ÄŽ€–S‚·‚銳ŽŅ‚Ŗ‘½‚¢B‚»‚Ģ‚½‚߉»Šw—Ć–@‚Ģ‹­“x‚šŽć‚߂邱‚Ę‚É‚ę‚čC‚—īŽŅAPL ‚ĢŽ”—Ɛ¬Ń‰ü‘P‚ŖŠś‘Ņ‚³‚ź‚éB

ƒXƒyƒCƒ“‚ĢPETHEMA ƒOƒ‹[ƒv‚ĶƒI[ƒ‹ƒgƒ‰ƒ“ƒXŒ^ƒŒƒ`ƒmƒCƒ“Ž_iATRAj‚ĘƒCƒ_ƒ‹ƒrƒVƒ“•¹—p‚ĢŠ°‰š“±“ü—Ć–@‚ĘƒAƒ“ƒgƒ‰ƒTƒCƒNƒŠƒ“ŒnR‚Ŗ‚ńÜ’†S‚Ģ’nŒÅ‚ß—Ć–@‚©‚ē‚Č‚éLPA96/99 Œ¤‹†‚š ”N—ī§ŒĄ‚Č‚µ‚ōs‚Į‚½B60 ĪˆČć‚ĢŠ°‰š—¦‚Ķ84“‚Ę—ĒD‚¾‚Į‚½‚ą‚Ģ‚ĢC‘ŠśŽ€–S‚ĢŒ“ˆö‚ĶŠ“õĒŽ€‚Ŗ‘½‚­CŠ°‰šŒć—Ć–@’†‚ĢŽ€–S‚Ķ71 ĪˆČć‚Å‚Ķ19“‚ĘŽį”NŽŅ‚É”ä‚׍‚—¦‚Å‚ ‚Į‚½1jBƒCƒ^ƒŠƒA‚ĢGIMEMA ƒOƒ‹[ƒv‚Ķ’nŒÅ‚ß—Ć–@3 ƒR[ƒX‚©‚ē‚Č‚éAIDA —Ć–@‚š60 ĪˆČć‚É‚Ķ1 ƒR[ƒX‚ÉŒø‚ē‚·‚±‚Ę‚É‚ę‚čCŽ”—ĆŠÖ˜A—LŠQŽ–Ū‚šŒø‚ē‚µC“Æ“™‚ĢŽ”—Ɛ¬Ń‚š“¾‚½2jB

ˆŸƒqŽ_iATOj‚Ķ”N—īˆĖ‘¶‚Ģ•›ģ—p‚Ŗ­‚Č‚­C‚—īŽŅAPL ‚É‘Ī‚µ‚Ä‚ąŠś‘Ņ‚³‚ź‚éB•Ä‘Intergroup C9710 Œ¤‹†‚Å‚ĶC’nŒÅ‚ß—Ć–@‚ÅATO ‚š’P“Ę‚Å’Ē‰Į‚µ‚½ŒQ‚Ķ61`79 Ī‚Ģ‚—īŽŅŒQ‚Å‚ą—LˆÓ‚Č—\Œć‚Ģ‰ü‘P‚š”F‚ß‚½3jBMD ƒAƒ“ƒ_[ƒ\ƒ“‚Ŗ‚ńƒZƒ“ƒ^[‚ĶCŠ°‰š“±“üE’nŒÅ‚ß—Ć–@‚ÉATO ‚ĘATRA ‚š•¹—p“Š—^‚µ‚½ŽŽŒ±‚šs‚¢C60 ĪˆČć‚Å‚ĢŠ°‰š—¦‚Ķ83“C10 —į’†9 —į‚ŖŠ°‰šˆŪŽiŠĻŽ@’†‰›ŠśŠŌ17 ƒJŒŽj‚Ę‚Ģ—ĒD‚ČŒ‹‰Ź‚š•ń‚µ‚Ä‚¢‚é4jB

ˆČć‚ę‚čC‚—īŽŅ‚É‚ĶC’Źķ‚ĢR‚Ŗ‚ńÜ‚ę‚č’v–½“I‚Č—LŠQŽ–Ū‚š‚«‚½‚·‚±‚Ę‚Ģ­‚Č‚¢ATO ƒx[ƒX‚ĢŽ”—Ć‚šs‚¤‚Ģ‚ą‘Ć“–‚ʍl‚¦‚ē‚ź‚éB

yŽQl•¶Œ£z

1j Sanz MA, et al. All-trans retinoic acid and anthracycline monochemotherapy for the treatment of elderly patients with acute promyelocytic leukemia. Blood. 2004 ; 104i12j : 3490-3.i1iiDiij

2j Mandelli F, et al. Treatment of elderly patients i„or60 yearsj with newly diagnosed acute promyelocytic leukemia. Results of the Italian multicenter group GIMEMA with ATRA and idarubicin iAIDAjprotocols. Leukemia. 2003 ; 17i6j : 1085-90.i1iiAj

3j Powell BL, et al. Arsenic trioxide improves event-free and overall survival for adults with acute promyelocytic leukemia : North American Leukemia Intergroup Study C9710. Blood. 2010 ; 116 i19j : 3751-7.i2Diij

4j Estey E, et al. Use of all-trans retinoic acid plus arsenic trioxide as an alternative to chemotherapy in untreated acute promyelocytic leukemia. Blood. 2006 ; 107i9j : 3469-73.i3iDivj


CQ10

”DP’†‚É”­Ē‚µ‚½APL ‚š‚Ē‚Ģ‚ꂤ‚ÉŽ”—Ć‚·‚ׂ«‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[4
”DP‰Šś‚É‚Ø‚¢‚āCŠ³ŽŅ‚Ŗ”DP’†ā‚ɓƈӂµ‚Č‚¢ź‡‚ĶCĆŠļ«‚Ģ‚‚¢ATRA ‚Ģ“Š—^‚š”š‚Æ‚éB

„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2A
”DP‚Ģ’†ŒćŠś‚É‚Ø‚¢‚Ä‚ĶCATRA ‚ąŠÜ‚߂čR‚Ŗ‚ńÜ‚Ģ“Š—^‚Ķ”äŠr“IˆĄ‘S‚Ę‚Č‚é‚ŖC—¬ŽY‚ā–¢nŽY‚ĢƒŠƒXƒN‚Ķ‚‚­‚Ȃ邱‚Ę‚©‚ēCŽ”—ĆƒN[ƒ‹‚Ģ‡ŠŌ‚ĢŒv‰ę“IoŽY‚šŒŸ“¢‚·‚éB

„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2B
ˆŸƒqŽ_‚Ķ”DP‚Ģ‚·‚ׂĂĢŠśŠŌ‚ÉŽg—p‚·‚ׂ«‚Å‚Ķ‚Č‚¢B

y‰š ąz

APL ‚ĶAML ‚Ģ’†‚Å‚ą”äŠr“IŽį”NŽŅ‚É”­Ē‚µC”DP‚ʍ‡•¹‚·‚é•p“x‚Ŗ‚‚¢BAPL ‚Ķ”­ĒŽž‚É’˜–¾‚ȏoŒŒŒXŒü‚š’ę‚·‚é‚Ę‚Ę‚ą‚ɁCƒI[ƒ‹ƒgƒ‰ƒ“ƒXŒ^ƒŒƒ`ƒmƒCƒ“Ž_iATRAj‚āˆŸƒqŽ_iATOj‚É‚Ķ‹­‚¢ĆŠļ«‚Ŗ‚ ‚é1j2jBATO ‚ĶC‘ŁŽ™‚É‘Ī‚·‚éd“Ä‚Č“Å«2j‚©‚ēC”DP‘SŠś‚Å“Š—^‚š”š‚Æ‚é•K—v‚Ŗ‚ ‚éB

”DP‰Šś‚ĶCR‚Ŗ‚ńÜŽ”—Ć‚É‚ę‚č‘ŁŽ™‚ɏd‘å‚ČŠļŒ`‚šˆų‚«‹N‚±‚·‰Ā”\«‚Ŗ‚‚¢B‚Ü‚½‘ŁŽ™Ž€–S‚Ģ‰Ā”\«‚ą‚‚­C•ź‘Ģ‚Ģ¶–½‚ĢˆĄ‘S‚š—Dę‚µC”DP’†ā‚š\•Ŗ‚ÉŒŸ“¢‚·‚é•K—v‚Ŗ‚ ‚éB‹“Ž™Šó–]‚Č‚Ē‚Ģ——R‚É‚ę‚č”DP’†ā‚šs‚ķ‚Č‚¢ź‡‚ĶCATRA ‚āATO ‚Ģ“Š—^‚Ķ”š‚ƁCƒAƒ“ƒgƒ‰ƒTƒCƒNƒŠƒ“’PÜ‚ÅŽ”—Ć‚šs‚¤‚±‚Ę‚Ŗ–]‚Ü‚µ‚¢3jB‚»‚Ģź‡CŽ‰—n«‚Ŗ‚‚­‘Ł”ÕˆŚs—¦‚Ģ‚‚¢ƒCƒ_ƒ‹ƒrƒVƒ“iIDRj‚ę‚č‚ąƒ_ƒEƒmƒ‹ƒrƒVƒ“iDNRj‚Ģ“Š—^‚Ģ•ū‚ŖD‚Ü‚µ‚¢‚Ę‚¢‚¤ˆÓŒ©‚Ŗ‚ ‚é4jBŠ®‘SŠ°‰š‚Ŗ“¾‚ē‚źC”DP’†Šś‚ɈŚsŒć‚ĶCATRA •¹—p‚ĢR‚Ŗ‚ńÜŽ”—Ć‚šs‚¤B

”DP’†EŒćŠś‚ĶCATRA ‚šŠÜ‚ŽRŠąÜŽ”—Ć‚šs‚Į‚Ä‚ąo¶Ž™‚ÉŠļŒ`‚ŖoŒ»‚·‚é‰Ā”\«‚Ķ”äŠr“I’į‚¢‚Ę‚³‚ź‚é5jB‚µ‚©‚µC—¬ŽY‚āC’į‘ĢdŽ™‚ĢoŽYEV¶Ž™D’†‹…Œø­ĒES‡•¹Ē‚Č‚Ē‚ĢƒŠƒXƒN‚Ķ‚‚­‚Č‚é3j‚½‚߁CS‹@”\•]‰æ‚Č‚Ē‘ŁŽ™‚Ģƒ‚ƒjƒ^ƒŠƒ“ƒO‚š•p‰ń‚©‚ĀŒµ–§‚ɍs‚¤•K—v‚Ŗ‚ ‚éB

ˆź”Ź‚É32 T‚š’“‚¦‚ź‚ĪV¶Ž™‰ČˆćŽt‚Ę‚Ģ˜AŒg‚Ģ‰ŗC‘ŽY‚³‚¹‚邱‚Ę‚Ŗ‰Ā”\‚Ę‚Č‚éB36 TˆČ‘O‚ĢoŽY‚Å‚ĶCV¶Ž™ŒÄ‹z‹‡”—ĒŒóŒQ‚É‚ę‚郊ƒXƒN’įŒø‚Ģ‚½‚߁CƒXƒeƒƒCƒh‚Ģ‘O“Š—^‚Ŗ–]‚Ü‚µ‚¢3jB

oŽYŒćCR‚Ŗ‚ńÜŽ”—Ć’†‚ĶŽö“ū‚š”š‚Æ‚éB‚Ü‚½CˆŪŽ—Ć–@’†‚Ģ”DP‚Ķ”š‚Æ‚é‚ꂤ‚ÉŽw“±‚·‚éB”DP‡•¹APL ‚Å’†āŽčp‚šs‚¤ź‡C‘Sgó‘Ō‚Ģˆ«‚¢ŽžŠś‚ɍs‚ķ‚ø‘Ņ‹@“I‚ɍs‚¤‚±‚Ę‚ą‘I‘šŽˆ‚Ę‚Č‚é‚ŖC‹Ł‹}Žčp‚Ŗ•K—v‚É‚Č‚éź‡‚ą‚ ‚éBŽY‰ČˆćC–ƒŒ‰ČˆćCV¶Ž™‰Čˆć‚ʏ\•Ŗ‚ɘAŒg‚šŽę‚ē‚Č‚Æ‚ź‚Ī‚Č‚ē‚Č‚¢B

yŽQl•¶Œ£z

1j Lammer EJ, et al. Retinoic acid embryopathy. N Engl J Med. 1985 ; 313i14j : 837-41.i3iiiCj

2j Golub MS, et al. Developmental and reproductive toxicity of inorganic arsenic : animal studies and human concerns. J Toxicol Environ Health B Crit Rev. 1998 ; 1i3j : 199-241.iƒŒƒrƒ…[j

3j Sanz MA, et al. Management of acute promyelocytic leukemia : recommendations from an expert panel on behalf of European Leukemia Net. Blood. 2009 ; 113i9j : 1875-91iƒKƒCƒhƒ‰ƒCƒ“j

4j Cardonick E, et al. Use of chemotherapy during human pregnancy. Lancet Oncol. 2004 ; 5i5j : 283-91.iƒŒƒrƒ…[j

5j Culligan DJ, et al. The management of acute promyelocytic leukemia presenting during pregnancy. Clin Leukemia. 2007 ; 1i3j : 183-91.i3iiiCj


3
  ‹}«ƒŠƒ“ƒp‰č‹…«”’ŒŒ/ƒŠƒ“ƒp‰č‹…«ƒŠƒ“ƒpŽī
iacute lymphoblastic leukemiaFlymphoblastic lymphomaFALL/LBLj

Ÿ‘˜_

ˆČ‘O‚ĶC‹}«”’ŒŒ•a‚Ęˆ««ƒŠƒ“ƒpŽī‚ĶC‚»‚ź‚¼‚źœ‘—R—ˆ‚ĘƒŠƒ“ƒpß—R—ˆ‚ĢŽīį‡‚ʍl‚¦‚ē‚źCœ ‘‚ɃŠƒ“ƒp‰č‹…‚ŖZ‚µ‚Ä‚¢‚鏼‡‚Ķ‹}«ƒŠƒ“ƒp‰č‹…«”’ŒŒ•aiacute lymphoblastic leukemiaF ALLjCœ‘‚Ö‚ĢZ‚Ŗ‚Č‚¢ź‡‚ĶƒŠƒ“ƒp‰č‹…«ƒŠƒ“ƒpŽīilymphoblastic lymphomaFLBLj‚Ę‚µ ‚Ä‚¢‚½B

WHO •Ŗ—Ži2008j‚Å‚ĶCƒŠƒ“ƒpŒnŽīį‡‚šB ×–EŒn‚ĘT/NK ×–EiT ×–E‚Ø‚ę‚Ńnatural killerFNK ×–EjŒn‚Ę‚É‘å•Ź‚µC³ķƒŠƒ“ƒpŒn×–E‚Ģ•Ŗ‰»’iŠK‚ĘŠTŠ‡‘Ī‰ž‚³‚¹‚čוŖ—Ž‚µ‚½i•\1j1jB‚·‚Č‚ķ‚æCALL/LBL ‚Ķ‘O‹ģB ×–Eiprecursor B-cellj—R—ˆ‚Ę‘O‹ģT ×–Eiprecursor T-cellj—R—ˆ‚É•Ŗ—Ž‚³‚źC‚»‚ź‚¼‚źCB ×–EƒŠƒ“ƒp‰č‹…«”’ŒŒ•a/ ƒŠƒ“ƒpŽī‚ĘT ×–EƒŠƒ“ƒp‰č‹…«”’ŒŒ•a/ƒŠƒ“ƒpŽī‚ĘŒÄ‚Ī‚źCB ×–EƒŠƒ“ƒp‰č‹…«”’ŒŒ•a/ƒŠƒ“ƒpŽī‚ĶC”ń“Į’čŒ^inot otherwise specifiedj‚Ę”½•œ«ˆā“`ŽqˆŁķ‚š”ŗ‚¤iwith recurrent genetic abnormalitiesjƒ^ƒCƒv‚É•Ŗ—Ž‚³‚ź‚é1jBˆČ‰ŗ‚Ģ•\‚ɁC”½•œ«ˆā“`ŽqˆŁķ‚š”ŗ‚¤B ×–EƒŠƒ“ƒp‰č‹…«”’ŒŒ•a/ƒŠƒ“ƒpŽī‚Ģ×•Ŗ—Ž‚ąŠÜ‚ß‚Ä‹LŚ‚·‚éB

FAB •Ŗ—Ž2j‚ĢL3 ‚ĶCWHO •Ŗ—Ž‚Å‚Ķ¬nB ×–EŽīį‡imature B-cell neoplasmsj‚Å‚ ‚éƒo[ƒLƒbƒgƒŠƒ“ƒpŽīiBurkitt lymphomaj‚É•ļŠÜ‚³‚źCALL/LBL ‚É‚ĶŠÜ‚Ü‚ź‚Ä‚¢‚Č‚¢1jB

—\ŒćˆöŽq‚ĶC”N—īC‰fŽž”’ŒŒ‹…”CŠ®‘SŠ°‰š‚Ü‚Å‚ĢŠśŠŌ‚Ø‚ę‚ŃPhiladelphiaiPhjõF‘Ģ‚Č‚¢‚µt (4;11)‚Å‚ ‚é3jBPhõF‘Ģ‚Ŗ”F‚ß‚ē‚ź‚émB lymphoblastic leukemia/lymphoma with t (9;22)(q34;q11.2) ; BCR-ABL1nź‡‚ĶCƒCƒ}ƒ`ƒjƒu‚š‘ć•\‚Ę‚·‚éƒ`ƒƒVƒ“ƒLƒi[ƒ[‘jŠQÜ‚Ģ—LŒų«‚Ŗ–¾‚ē‚©‚É‚Č‚Į‚Ä‚¢‚é‚Ģ‚ŁCŽ”—Ć‚Ģ‘I‘š‚É‚ĶCPh õF‘Ģ‚Č‚¢‚µBCR-ABL1 —Z‡ˆā“`Žq‚š’Tõ‚·‚邱‚Ę‚Ŗd—v‚Å‚ ‚éB

•\1@ALL/LBL ‚ĢWHO •Ŗ—Ži2008j
  • B lymphoblastic leukemia/lymphoma, not otherwise specified
  • B lymphoblastic leukemia/lymphoma with recurrent genetic abnormalities

    B lymphoblastic leukemia/lymphoma with t (9;22)(q34;q11.2); BCR-ABL1
    B lymphoblastic leukemia/lymphoma with t (v;11q23); MLL rearrnged
    B lymphoblastic leukemia/lymphoma with t (12;21)(p13;q22); TEL-AML1iETV6-RUNX1j
    B lymphoblastic leukemia/lymphoma with hyperdiploidy
    B lymphoblastic leukemia/lymphoma with hypodiploidyiHypodiploid ALLj
    B lymphoblastic leukemia/lymphoma with t (5;14)(q31;q32); IL3-IGH
    B lymphoblastic leukemia/lymphoma with t (1;19)(q23;p13.3); E2A/PBX1iTCF3-PBX1j

  • T lymphoblastic leukemia/lymphoma
yŽQl•¶Œ£z

1j Swerdlow SH, et al. eds. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon, IARC ; 2008.

2j Bennett JM, et al. : The morphological classification of acute lymphoblastic leukaemia : Concordance among observers and clinical correlations. Br J Haematol. 1981 ; 47i4j : 553-61.

3j ’|“ą@mD‹}«ƒŠƒ“ƒp«”’ŒŒ•aDŒŒ‰tź–åˆćƒeƒLƒXƒgD“ś–{ŒŒ‰tŠw‰ļ•ŅC“ģ]“°C“Œ‹žC2011D



ŸƒAƒ‹ƒSƒŠƒYƒ€

i¦jCQ”Ō†iƒsƒ“ƒNF•”•Ŗj‚šƒNƒŠƒbƒN‚·‚é‚ʁC‰šą‰ę–Ź‚ÖˆŚ“®‚µ‚Ü‚·

‹}«ƒŠƒ“ƒp‰č‹…«”’ŒŒ•a/ ƒŠƒ“ƒp‰č‹…«ƒŠƒ“ƒpŽī‚ĢƒAƒ‹ƒSƒŠƒYƒ€ CQ1 CQ2 CQ3 CQ4 CQ5 CQ8 CQ6 CQ7 CQ9`11 CQ12 CQ13 CQ14

LBL ‚ĶCWHO •Ŗ—Ž‚Å‚ĶALL ‚Ę“Æ‚¶ƒJƒeƒSƒŠ[‚É‘®‚µCALL ‚Ę“Æ‚¶Ž”—Ć‚Ŗ„§‚³‚ź‚éiCQ1jB

Š°‰š“±“üŽ”—Ć‚Ģ‘I‘š‚ĶC‚Ü‚øPhiladelphiaiPhjõF‘Ģ‚Ģ—L–³‚Å•Ŗ—Ž‚µCPh —z«‚Å‚ ‚ź‚ĪƒCƒ}ƒ`ƒjƒu‚šŠÜ‚ŽŽ”—Ć‚Ŗ„§‚³‚ź‚éiCQ2jBPh ‰A«‚Ģź‡CŽvtŠśEŽį”N¬li‚؂؂Ž‚Ė30 Ī‚܂Łj‚Å‚ ‚ź‚Ī¬Ž™ƒvƒƒgƒR[ƒ‹‚Ŗ„§‚³‚ź‚é‚ŖiCQ3jC‚—īŽŅi‚؂؂Ž‚Ė55`60 ĪˆČćj‚āˆź”Ź¬l‚Ģź‡‚ĢŠm—§‚³‚ź‚½Ž”—Ć–@‚Ķ‚Č‚¢iCQ4, 5jBƒ}[ƒJ[‚É‚ę‚é‘I‘š‚ÉŠÖ‚µ‚Ä‚ĶCT ×–E«ALLiT-ALLj‚ĘB ×–E«ALLiB-ALLj‚ňŁ‚Č‚Į‚½Ž”—Ć‚šs‚¤‚ׂ«‚Å‚ ‚é‚Ę‚Ģ–¾Šm‚ȍŖ‹’‚Ķ‚Č‚¢iCQ6jBƒvƒŒƒhƒjƒ]ƒƒ“iPSLj‚Ģ‘O“Š—^‚ĶC¬Ž™‚Å‚Ķˆź”Ź“I‚Å‚ ‚čC¬lALL ‚Å‚ąPSL ‘O“Š—^‚Ģ”½‰ž«‚š‚Ż‚邱‚Ę‚ÉˆÓ‹`‚Ķ‚ ‚é‚ŖC‘w•Ź‰»‚ĢŽw•W‚Ę‚Č‚č“¾‚é‚©‚Ē‚¤‚©‚Ķ‚Ü‚¾–¾‚ē‚©‚Å‚Ķ‚Č‚¢iCQ7jB

Š®‘SŠ°‰šicomplete remissionFCRj‚É“ž’B‚·‚ź‚ĪCPh õF‘Ģ‚Ģ—L–³‚ā”N—ī‚É‚©‚©‚ķ‚ē‚øC‰»Šw—Ć–@Ü‚É‚ę‚é’†•_ŒoŒnicentral nervous systemFCNSjÄ”­—\–h‚Ķ•s‰ĀŒ‡‚Å‚ ‚é‚ŖC‘S”]ĘŽĖ‚Ģ“K‰ž‚ĶŒĄ’č“I‚Å‚ ‚éiCQ8jBCR Žž‚ ‚é‚¢‚Ķ‚»‚ĢŒć‚ĢŒo‰ß‚Å‚Ģ”÷¬Žc‘¶•a•Ļiminimal residual diseaseFMRDj‚ĢŠĻŽ@‚Ķ—LˆÓ‹`‚Å‚ ‚é‚ŖiCQ9jC¬lALL ‚É‚Ø‚Æ‚éMRD ‚É‚ę‚é‘w•Ź‰»Ž”—Ć‚Ģ¬Ń‚Ķ–¾‚ē‚©‚Å‚Ķ‚Č‚¢BŠ°‰šŒć—Ć–@‚Ę‚µ‚Ä‘å—ŹƒLƒƒTƒCƒh‚ā‘å—ŹƒƒgƒgƒŒƒLƒT[ƒgiMTXj‚Ķ“KŲ‚ČŽ”—Ć‘I‘šŽˆ‚Å‚ ‚é‚ŖiCQ10jCŽ©‰Ę‘¢ŒŒŠ²×–EˆŚA•¹—p‘å—Ź‰»Šw—Ć–@‚Ķs‚¤‚ׂ«‚Å‚Ķ‚Č‚¢iCQ11jBŽ”—Ć‘O‚ɏcŠu•a•Ļ‚Ģ”F‚ß‚ē‚ź‚éT ×–E«LBLiT-LBLj‚É‘Ī‚·‚écŠuĘŽĖ‚Ķ‹ĒŠÄ”­—\–h–Ś“I‚Å‚ ‚é‚ŖC•K‚ø‚µ‚ą¬Œ÷‚µ‚Ä‚¢‚é‚Ę‚ĶŒ¾‚¢“ļ‚­CcŠuĘŽĖ‚šŠÜ‚Ü‚Č‚¢Ž”—Ć‚ą‘Ć“–‚Å‚ ‚éiCQ12jB

Ph —z«Ē—į‚¾‚Æ‚Å‚Ķ‚Č‚­Ph ‰A«‚Å‚ ‚Į‚Ä‚ąCHLA ˆź’v“K‡ƒhƒi[‚Ŗ‚¢‚ź‚ĪC‘ęˆźCR Šś‚Ģ“ÆŽķ‘¢ŒŒŠ²×–EˆŚA‚Ķ„§‚³‚ź‚éiCQ13jB‚Ü‚½CŒøŽć‘Oˆ’u‚É‚ę‚é‘¢ŒŒŠ²×–EˆŚA‚ąŽŽ‚Ż‚é‚É’l‚·‚éiCQ14jB

Ä”­‚µ‚½ź‡Ž”—Ć‚ĶC‘OŽ”—Ć—š‚ĘÄ”­‚Ü‚Å‚ĢŠśŠŌ‚šl—¶‚µ‚ÄŽ”—Ć–ņÜ‚š‘I‘š‚µCĒ—į‚É‚ę‚Į‚Ä‚ĶV‹K–ņÜ‚ĢŽg—p‚ąl—¶‚·‚éiCQ15jB



CQ1

œ‘Z‚Ģ‚Č‚¢ƒŠƒ“ƒp‰č‹…«ƒŠƒ“ƒpŽī‚ĢŽ”—Ć‚ĶALL ‚Ę“Æ‚¶Ž”—Ć‚ŖŠ©‚ß‚ē‚ź‚é‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2B
œ‘Z‚Ģ—L–³‚É‚©‚©‚ķ‚ē‚øCƒŠƒ“ƒp‰č‹…«ƒŠƒ“ƒpŽī‚ĢŽ”—Ć‚ĶALL ‚Ę“Æ—l‚ɍs‚¤‚±‚Ę‚Ŗ„§‚³‚ź‚éBicŠu•a•Ļ‚š—L‚·‚鏼‡‚Ģ‘Ī‰ž‚É‚Ā‚¢‚Ä‚ĶCCQ11 ŽQĘj

y‰š ąz

LBL ‚ĶC¶•ØŠw“I‚É‚ĶALL ‚Ę“Æˆź‚ĢŽ¾Š³‚Å‚ ‚čCœ‘Z‚ĢŠ„‡‚Ŗ25“–¢–ž‚Ģź‡‚ÉLBL ‚ʐf’f‚³‚ź‚éBLBL ‚Ģ–ń80“ˆČć‚ĶT ×–E«LBLiT-LBLj‚Ŗč‚߁CŽį”N’j«CcŠu•a•Ļ‚š—L‚·‚邱‚Ę‚Ŗ‘½‚­Cœ‘Z‚š”F‚ß‚Č‚¢iœ‘‰č‹…”ä—¦‚Ŗ5“–¢–žjĒ—į‚ĢŠ„‡‚Ķ36`85“‚Ę•ń‚³‚ź‚Ä‚¢‚é1j`4jBˆź•ūC­”—į‚ĢŒŸ“¢‚Å‚ ‚é‚ŖCB ×–E«LBLiB-LBLj‚ĶŽį”N—«CßŠO•a•Ļi“Į‚É”ē•†•a•Ļj‚š—L‚µCœ‘Z‚š”F‚ß‚Č‚¢Ē—į‚Ŗ‘½‚¢‚±‚Ę‚Ŗ•ń‚³‚ź‚Ä‚¢‚é5j`7jB

‚Ü‚½CALL Ž”—ĆƒŒƒWƒƒ“‚Ģ“K—p‚É‚ę‚čCLBL ‚ĢŽ”—Ɛ¬Ń‚Ķ”ņ–ō“I‚ÉŒüć‚µ‚½‚±‚Ę‚Ŗ•ń‚³‚ź‚Ä‚¢‚é‚Ŗ1jCƒ‰ƒ“ƒ_ƒ€‰»”äŠrŽŽŒ±‚É‚ę‚éƒGƒrƒfƒ“ƒX‚Ķ‚Č‚¢B

¬lT-LBL 45 —į‚š‘ĪŪ‚Ę‚µ‚½ƒhƒCƒc‚Ģ‘½Ž{Ż‹¤“ÆŒć•ūŽ‹“IŒ¤‹†‚Å‚ĶCALL ƒŒƒWƒƒ“‚šŽ{s‚µC7 ”N‘S¶‘¶Š„‡iOSj‚Ø‚ę‚Ń–³•a¶‘¶Š„‡iDFSj‚Ķ‚»‚ź‚¼‚ź51“C62“‚Å‚ ‚Į‚½‚±‚Ę‚š•ń‚µ‚½1jB‚Ü‚½C¬lLBL 33 —į‚š‘ĪŪ‚Ę‚µ‚½CMD ƒAƒ“ƒ_[ƒ\ƒ“‚Ŗ‚ńƒZƒ“ƒ^[‚Ģ’PŽ{ŻŒć•ūŽ‹“IŒ¤‹†‚Å‚ĶChyper-CVAD/MA —Ć–@iCPA, VCR, DXR, DEXC‚—p—ŹMTXC‚—p—ŹAraCj‚šŽ{s‚µC3 ”N–³‘ˆ«¶‘¶Š„‡iPFSj‚Ø‚ę‚ŃOS ‚Ķ‚»‚ź‚¼‚ź66“C70“‚Å‚ ‚Į‚½2jB‚±‚ź‚ē2 •ń‚É‚Ø‚¢‚āCœ‘Z‚Ģ—L–³‚Ķ—\ŒćˆöŽq‚É‚Ķ‚Č‚ē‚Č‚©‚Į‚½B

yŽQl•¶Œ£z

1j Hoelzer D, et al. Outcome of adult patients with T-lymphoblastic lymphoma treated according to protocols for acute lymphoblastic leukemia. Blood. 2002 ; 99i12j : 4379-85.i3iiiAj

2j Thomas DA, et al. Outcome with the hyper-CVAD regimens in lymphoblastic lymphoma. Blood. 2004 ; 104i6j : 1624-30.i3iiiAj

3j Hunault M, et al. Outcome of adult T-lymphoblastic lymphoma after acute lymphoblastic leukemia-type treatment : a GOELAMS trial. Haematologica. 2007 ; 92i12j : 1623-30.i3iiiDivj

4j Le Gouill S, et al. Adult lymphoblastic lymphoma : a retrospective analysis of 92 patients under 61 years included in the LNH87/93 trials. Leukemia. 2003 ; 17i11j : 2220-4.i3iiiDivj

5j Soslow RA, et al. B-lineage lymphoblastic lymphoma is a clinicopathologic entity distinct from other histologically similar aggressive lymphomas with blastic morphology. Cancer. 1999 ; 85 i12j : 2648-54.i3iiiDivj

6j Lin P, et al. Precursor B-cell lymphoblastic lymphoma : a predominantly extranodal tumor with low propensity for leukemic involvement. Am J Surg Pathol. 2000 ; 24i11j : 1480-90.i3iiiDivj

7j Maitra A, et al. Precursor B-cell lymphoblastic lymphoma. A study of nine cases lacking blood and bone marrow involvement and review of the literature. Am J Clin Pathol. 2001 ; 115i6j : 868-75.i3iiiDivj


CQ2

Ph —z«ALL ‚É‘Ī‚µ‚ă`ƒƒVƒ“ƒLƒi[ƒ[‘jŠQÜ‚Ķ‚Ē‚Ģ–ņÜ‚š‚Ē‚Ģ‚ꂤ‚ÉŽg‚¤‚ׂ«‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2A
Ph —z«ALL ‚ĢŠ°‰š“±“ü—Ć–@C’nŒÅ‚ß—Ć–@‚É‚Ø‚¢‚ăCƒ}ƒ`ƒjƒu‚š•¹—p‚·‚邱‚Ę‚É‚ę‚čC‚ę‚č‚‚¢Š®‘SŠ°‰š—¦‚ʐ¶‘¶Š„‡‚ŖŠś‘Ņ‚Å‚«C„§‚³‚ź‚éB

y‰š ąz

¬l‚ĢPh —z«ALL ‚É‘Ī‚·‚鏉‰ńŽ”—Ć‚É‚Ø‚Æ‚éƒ`ƒƒVƒ“ƒLƒi[ƒ[‘jŠQÜiTKIj‚ĢŽg—p‚É‚Ā‚¢‚Ä‹Lq‚·‚éB‚—īŽŅ‚ĢPh —z«ALL ‚ÉŠÖ‚µ‚Ä‚Ķ‚—īŽŅ‚Ģ€iCQ4j‚šCˆŚA‚É‚Ø‚Æ‚éTKI ‚ĢŽg—p‚ÉŠÖ‚µ‚Ä‚ĶˆŚA‚Ģ€iCQ10j‚šŽQĘ‚³‚ź‚½‚¢B

Ph —z«ALL ‚Ģ‰‰ńŽ”—Ć‚É‚Ø‚Æ‚éTKI ‚ĢŽg—p–@‚É‚Ā‚¢‚ÄŒŸ“¢‚µ‚½‘ęⅢ‘ŠŽŽŒ±‚Ķ‘¶Ż‚µ‚Ä‚¢‚Č‚¢Bˆź•ūCƒCƒ}ƒ`ƒjƒu‚š—p‚¢‚½‘ęⅡ‘ŠŽŽŒ±‚Ķ7 ŽŽŒ±‚Ŗ˜_•¶‚Ę‚µ‚Ä”­•\‚³‚ź‚Ä‚¢‚é1j`7jB‚¢‚ø‚ź‚ąŠ°‰š“±“ü—Ć–@C’nŒÅ‚ß—Ć–@CƒCƒ}ƒ`ƒjƒu‚ĢŽg—p•ū–@‚Ŗˆź’č‚Å‚Ķ‚Č‚¢‚½‚߁C‚Ē‚ĢŽ”—Ć–@‚Ŗ‚ę‚¢‚©‚Ķ”»’肵‚Ŗ‚½‚¢‚ŖCŠ®‘SŠ°‰šiCRj—¦‚Ķ90“ˆČćCPCR ‚Ģ‰A«—¦‚ą’č‹`‚Ģ·‚Ķ‚ ‚é‚ą‚Ģ‚Ģ38`71“‚Ę•ń‚³‚ź‚Ä‚¢‚éB

‚³‚ē‚ɁC‚Ē‚Ģ•ń‚É‚Ø‚¢‚Ä‚ą‘ęˆźŠ°‰šŠś‚Ģ50`79“‚ĢĒ—į‚Å“ÆŽķ‘¢ŒŒŠ²×–EˆŚA‚Ŗs‚ķ‚ź‚Ä‚Ø‚čC¶‘¶Š„‡‚Ģ”äŠrŒŸ“¢‚Ŗ¢“ļ‚Ę‚Č‚Į‚Ä‚¢‚éB

‚¢‚ø‚ź‚É‚µ‚Ä‚ąLALA-94 8j‚É‘ć•\‚³‚ź‚éTKI ‚š—p‚¢‚Č‚¢‰»Šw—Ć–@‚É”ä‚ׁChistorical control ‚Å‚Ķ‚ ‚é‚ą‚Ģ‚ĢC‚‚¢CR —¦‚ʐ¶‘¶Š„‡‚šŽ¦‚µ‚Ä‚Ø‚čC‚»‚ĢŽg—p‚Ŗ„§‚³‚ź‚éB

ƒCƒ}ƒ`ƒjƒuˆČŠO‚ĢTKI ‚ÉŠÖ‚µ‚Ä‚ĶCƒ_ƒTƒ`ƒjƒu‚š—p‚¢‚½‘ęⅡ‘ŠŽŽŒ±‚Ŗ2 ŽŽŒ±•ń‚³‚ź‚Ä‚Ø‚čC”Œć‚ĢŒŸ“¢Œ‹‰Ź‚Ŗ‘Ņ‚½‚ź‚é9j10jB

yŽQl•¶Œ£z

1j de Labarthe A, et al ; Group for Research on Adult Acute Lymphoblastic Leukemia iGRAALLj. Imatinib combined with induction or consolidation chemotherapy in patients with de novo Philadelphia chromosome-positive acute lymphoblastic leukemia : results of the GRAAPH-2003 study. Blood. 2007 ; 109i4j: 1408-13.i3iiiDivj

2j Wassmann B, et al. Alternating versus concurrent schedules of imatinib and chemotherapy as front-line therapy for Philadelphia-positive acute lymphoblastic leukemiaiPh{ALLj. Blood. 2006 ; 108i5j : 1469-77.i2Divj

3j Ribera JM, et al ; Programa Español de Tratamiento en Hematología ; Grupo Español Trasplante Hemopoyético Groups. Concurrent intensive chemotherapy and imatinib before and after stem cell transplantation in newly diagnosed Philadelphia chromosome-positive acute lymphoblastic leukemia. Final results of the CSTIBES02 trial. Haematologica. 2010 ; 95i1j : 87-95.i3iiiDivj

4j Yanada M, et al ; Japan Adult Leukemia Study Group. High complete remission rate and promising outcome by combination of imatinib and chemotherapy for newly diagnosed BCR-ABL-positive acute lymphoblastic leukemia : a phase Ⅱ study by the Japan Adult Leukemia Study Group. J Clin Oncol. 2006 ; 24i3j : 460-6.i3iiiDivj

5j Lee KH, et al. Clinical effect of imatinib added to intensive combination chemotherapy for newly diagnosed Philadelphia chromosome-positive acute lymphoblastic leukemia. Leukemia. 2005 ; 19i9j : 1509-16.i3iiiDivj

6j Thomas DA, et al. Treatment of Philadelphia chromosome-positive acute lymphocytic leukemia with hyper-CVAD and imatinib mesylate. Blood. 2004 ; 103i12j : 4396-407.i3iiiDivj

7j Bassan R, et al. Chemotherapy-phased imatinib pulses improve long-term outcome of adult patients with Philadelphia chromosome-positive acute lymphoblastic leukemia : Northern Italy Leukemia Group protocol 09/00. J Clin Oncol. 2010 ; 28i22j : 3644-52.i3iiiAj

8j Dombret H, et al ; Groupe dfEtude et de Traitement de la Leucémie Aiguë Lymphoblastique de lfAdulteiGET-LALA Groupj. Outcome of treatment in adults with Philadelphia chromosome-positive acute lymphoblastic leukemia--results of the prospective multicenter LALA-94 trial. Blood. 2002 ; 100i7j : 2357-66.i3iiiDivj

9j Ravandi F, et al. First report of phase 2 study of dasatinib with hyper-CVAD for the frontline treatment of patients with Philadelphia chromosome-positiveiPh{j acute lymphoblastic leukemia. Blood. 2010 ; 116i12j : 2070-7.i3iiiDivj

10j Foa R, et al ; GIMEMA Acute Leukemia Working Party. Dasatinib as first-line treatment for adult patients with Philadelphia chromosome-positive acute lymphoblastic leukemia. Blood. 2011 ; 118i25j : 6521-8.i3iiiDivj


CQ3

ŽvtŠśEŽį”N¬lALL ‚Ķ¬Ž™ƒvƒƒgƒR[ƒ‹‚Å‚ĢŽ”—Ć‚Ŗ„§‚³‚ź‚é‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2A
ŽvtŠśEŽį”N¬lALL ‚ĶC¬Ž™ƒvƒƒgƒR[ƒ‹‚É‚ę‚鎔—Ć‚ŖŠ©‚ß‚ē‚ź‚éB

y‰š ąz

16`20 Ī‚Ģ–¢Ž”—ĆALL Š³ŽŅ‚Å‚Ģ¬Ž™ƒvƒƒgƒR[ƒ‹iChildrenfs Cancer GroupFCCGj‚Ģ¬Ń‚ʐ¬lƒvƒƒgƒR[ƒ‹iCancer and Leukemia Group BFCALGBj‚Ģ¬Ń‚šŒć•ūŽ‹“I‚É”äŠr‚µCŠ®‘SŠ°‰šiCRj—¦‚Ķ90“‚Ę“Æ‚¶‚Å‚ ‚Į‚½‚ŖC7 ”N‘S¶‘¶Š„‡iOSj‚Ķ‚»‚ź‚¼‚ź67“‚Ę46“‚Å—LˆÓ‚ÉCCG ‚Ŗ—D‚ź‚Ä‚¢‚邱‚Ę‚Ŗ•ń‚³‚ź‚½B‚»‚Ģ——R‚Ę‚µ‚āCƒXƒeƒƒCƒhCƒrƒ“ƒNƒŠƒXƒ`ƒ“iVCRjCL-ƒAƒXƒpƒ‰ƒMƒi[ƒ[iL-Aspj‚Č‚Ē‚Ģ–ņÜ—Ź‚Ŗ‘½‚¢‚±‚Ę‚Ę’†•_ŒoŒn”’ŒŒ•a—\–h‚Ŗ•p‰ń‚Å‚ ‚邱‚Ę‚ŖŽw“E‚³‚ź‚½1jBSweden ‚Å‚ąC¬Ž™ƒvƒƒgƒR[ƒ‹36 —įi15`18 ĪC’†‰›’l15.6 Īj‚ʐ¬lƒvƒƒgƒR[ƒ‹23 —įi15`20 ĪC’†‰›’l18.2 Īj‚šŒć•ūŽ‹“I‚É”äŠr‚µC5 ”N–³ƒCƒxƒ“ƒg¶‘¶Š„‡iEFSj‚Ķ‘OŽŅ‚Ŗ—D‚ź‚Ä‚¢‚½i74“ vs 39“Cpƒ0.01j‚ŖChigh risk Ē—į‚Ŗ¬lƒvƒƒgƒR[ƒ‹ŒQ‚É‘½‚¢‚Ę‚¢‚¤ƒoƒCƒAƒXi14“ vs 39“Cp0.03j‚Ŗ‘¶Ż‚µ‚Ä‚¢‚½2jB

¬Ž™‚ʐ¬lƒvƒƒgƒR[ƒ‹‚š”äŠr‚µ‚½‘O•ūŽ‹“IŒ¤‹†‚Ķ‚Č‚¢‚ŖC¬Ž™ƒvƒƒgƒR[ƒ‹iPETHEMA ALL96j‚š•W€risk ‚ĢŽvtŠśi15`18 ĪCn35j‚Ø‚ę‚ŃŽį”N¬li19`30 ĪCn46jALL ‚ɍs‚¢CCR —¦98“C6 ”NEFS 61“C6 ”NOS 68“‚Ę—ĒD‚ŁCŽvtŠś‚ĘŽį”N¬lŠŌ‚ɍ·‚Ķ‚Č‚©‚Į‚½3jB

¬Ž™ƒvƒƒgƒR[ƒ‹‚š60 Ī‚Ü‚Å‚Ģ¬l‚É“K—p‚µ‚½•ń‚Å‚ĶCCR —¦89`93.5“C‘S—į‚ĢOS —¦‚ą60`63“‚ʏ]—ˆ‚Ģ¬Ń‚ę‚č—ĒD‚Å‚ ‚Į‚½4j5jBˆź•ūCŠ°‰š“±“ü’†‚ĢŽ€–S—¦‚Ķ45 ĪˆČć‚ĢĒ—į‚Å13“i45 Ī–¢–ž‚Ķ4“j4jC50 ĪˆČć‚Å20“ 5j‚Å‚ ‚čC’ˆÓ‚Ŗ•K—v‚Å‚ ‚éB

ˆČć‚©‚ē¬Ž™ƒvƒƒgƒR[ƒ‹‚ĶCŽvtŠśEŽį”N¬l‚Å‚Ķ—ĒD‚Ȑ¬Ń‚Å‚ ‚čCŽ€–S—¦‚ą‹–—e”ĶˆĶ‚ʍl‚¦‚ē‚ź‚éB

Japan Adult Leukemia Study GroupiJALSGj ALL202-U Œ¤‹†‚Å‚ĶC15`24 Ī‚Ü‚Å‚ĢBCRABL ‰A«ALL ‚š‘ĪŪ‚ɏ¬Ž™”’ŒŒ•aŒ¤‹†‰ļiJapan Association of Childhood Leukemia StudyFJACLSj‚Ę“Æ‚¶Ž”—Ć‚šs‚Į‚½6jBƒvƒŒƒhƒjƒ]ƒƒ“‚Ģ‘O“Š—^CŠ°‰š“±“ü—Ć–@‚Å‚ĢL-Asp ‚Ģ‘—Ź‚Ę’nŒÅ‚ß—Ć–@‚Å‚ĢL-Asp ‚ĘƒƒgƒgƒŒƒLƒT[ƒgiMTXj‚Ģ‘—Ź‚Ŗ“Į’„‚Å‚ ‚Į‚½BCR —¦‚Ķ94“i121/134 —įj‚ŁCALL97 ‚Å‚Ģ“Æ”N‘ć‚Ģ¬ŃiCR —¦84“j‚ɔ䂵—LˆÓ‚É—D‚ź‚Ä‚¢‚½ipƒ0.01jB

yŽQl•¶Œ£z

1j Stock W et al. What determines the outcomes for adolescents and young adults with acute lymphoblastic leukemia treated on cooperative group protocols? A comparison of Childrenfs Cancer Group and Cancer and Leukemia Group B studies. Blood. 2008 ; 112i5j : 1646-54.i3iiiAj

2j Hallböök H et al. Treatment outcome in young adults and children„10 years of age with acute lymphoblastc leukemia in Sweden. Cancer. 2006 ; 107i7j : 1551-61.i3iiiDij

3j Ribera JM et al. Comparison of the results of the treatment of adolescents and young adults with standard- risk acute lymphoblastic leukemia with the program Español de Tratamiento en Hematología pediatric-based protocol ALL-96. J Clin Oncol. 2008 ; 26i11j : 1843-9.i3iiiAj

4j Huguet F et al : Pediatric-inspired therapy in adults with Philadelphia chromosome-negative acute lymphoblastic leukemia : the GRAALL-2003 study. J Clin Oncol. 2009 ; 27i6j : 911-8.i3iiAj

5j Storring JM et al. Treatment of adults with BCR-ABL negative acute lymphoblastic leukaemia with a modified paediatric regimen. Br J Haematol. 2009 ; 146i1j : 76-85.i3iiAj

6j ‹ß“”‰p¶FŽvtŠśEŽį”N¬l‹}«ƒŠƒ“ƒp«”’ŒŒ•a‚š‘ĪŪ‚Ę‚µ‚½‰»Šw—Ć–@‚ĢˆĄ‘S«‚ĢŒŸ“¢FJALSG ALL202-U ‚Ģ’†ŠŌ‰šĶD—Տ°ŒŒ‰tD2012 ; 53i8j : 747-52.i3iCj


CQ4

‚—īŽŅALL ‚ĢŽ”—Ć–@‚Ķ‰½‚ŖŠ©‚ß‚ē‚ź‚é‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2B
‚—īŽŅ‚ĢPh —z«ALL ‚É‘Ī‚µ‚Ä‚ĶƒCƒ}ƒ`ƒjƒu‚Ģ•¹—p‚É‚ę‚芰‰š—¦‚Ģ‰ü‘P‚ŖŠś‘Ņ‚Å‚«‚邽‚߁CŽg—p‚Ŗ„§‚³‚ź‚éBPh ‰A«ALL ‚É‘Ī‚·‚鎔—Ć–@‚ĶŠm—§‚³‚ź‚Ä‚¢‚Č‚¢B

y‰š ąz

‚—īŽŅ‚Ģ’č‹`‚Ķ˜_•¶‚É‚ę‚Į‚ĈŁ‚Č‚é‚ą‚Ģ‚ĢC55 ‚ ‚é‚¢‚Ķ60 ĪˆČć‚ʍl‚¦‚é‚ʁC35`10 ”N‘O‚Ü‚Å‚ÉŽ”—Ć‚šŽó‚Æ‚½Š³ŽŅ‚Ģ¬Ń‚š‚Ż‚Ä‚ą‚—īŽŅ‚Ģ¬Ń‚É‚Ķ”N‘ć‚É‚ę‚é•Ļ‰»‚Ŗ”F‚ß‚ē‚ź‚Ä‚¢‚Č‚¢1jBŠ®‘SŠ°‰šiCRj—¦‚Ķ30`50 Ī‘ä‚ĢŠ³ŽŅ‚É”ä‚ׁC80“ vs 60“‚Ę’į‚­C3 ”N‘S¶‘¶Š„‡iOSj‚ą35“ vs 10“‚Ę•s—Ē‚Å‚ ‚é2jB

‚—īŽŅALL ‚Ģ‰‰ńŽ”—Ć‚ÉŠÖ‚·‚é—Տ°ŽŽŒ±‚ą­‚Č‚­CCD20 —z«‚ĢALL ‚É‘Ī‚·‚郊ƒcƒLƒVƒ}ƒuiRj‚šŠ°‰š“±“ü‚©‚ē•¹—p‚·‚é‘ęⅡ‘ŠŽŽŒ±3j4j‚ʁCPh —z«ALL ‚É‘Ī‚·‚éƒCƒ}ƒ`ƒjƒu‚š—p‚¢‚½1 ‚Ā‚Ģ‘ęⅢ‘ŠŽŽŒ±5j‚Ę2 ‚Ā‚Ģ‘ęⅡ‘ŠŽŽŒ±6j7j‚Ŗ•ń‚³‚ź‚Ä‚¢‚é‚Ģ‚Ż‚Å‚ ‚éB

R ‚ĶCD20 —z«‚ĢB ×–E«ALLiB-ALLj‚ ‚é‚¢‚Ķƒo[ƒLƒbƒgƒŠƒ“ƒpŽī‚É—p‚¢‚ē‚źC‘OŽŅ‚Å‚Ķhistorical control ‚É”ä‚׌ų‰Ź‚Ŗ”F‚ß‚ē‚ź‚øCŒćŽŅ‚Å‚ĶŒų‰Ź‚š”F‚ß‚é‚ą‚Ģ‚Ģ‘ĪŪ‚Ķ9 —į‚Å‚ ‚čC‚³‚ē‚Č‚éŒŸ“¢‚Ŗ•K—v‚Å‚ ‚é3j4jB

Ph —z«ALL ‚É‘Ī‚·‚éƒCƒ}ƒ`ƒjƒu‚ĢŒŸ“¢‚Ķ‰»Šw—Ć–@‚Ę‚Ģ”äŠr‚Ŗ‘ęⅢ‘ŠŽŽŒ±‚Ę‚µ‚Ä1 ŽŽŒ±s‚ķ‚źC—LˆÓ‚ČŠ°‰š—¦‚ĢŒüć‚š”F‚ß‚½‚ŖC¶‘¶Š„‡‚Ģ‰ü‘P‚Ķ”F‚ß‚ē‚ź‚Ä‚¢‚Č‚¢5jB2 ‚Ā‚Ģ‘ęⅡ‘ŠŽŽŒ±‚É‚Ø‚¢‚Ä‚ĶŽ”—Ć•ū–@‚Ŗ‚»‚ź‚¼‚źˆŁ‚Č‚é‚ą‚Ģ‚ĢC72`100“‚ĢCR —¦‚š•ń‚µ‚Ä‚Ø‚čC‚³‚ē‚ÉŒŸ“¢‚Ŗ•K—v‚ʍl‚¦‚ē‚ź‚éB

yŽQl•¶Œ£z

1j Pulte D, et al. Improvement in survival in younger patients with acute lymphoblastic leukemia from the 1980s to the early 21st century. Blood. 2009 ; 113i7j : 1408-11.iƒŒƒrƒ…[j

2j Larson RA. Acute lymphoblastic leukemia : older patients and newer drugs. Hematology Am Soc Hematol Educ Program. 2005 ; 131-136.iƒŒƒrƒ…[j

3j Thomas DA, et al. Chemoimmunotherapy with a modified hyper-CVAD and rituximab regimen improves outcome in de novo Philadelphia chromosome-negative precursor B-lineage acute lymphoblastic leukemia. J Clin Oncol. 2010 ; 28i24j : 3880-9.i3iiiDivj

4j Thomas DA, et al. Chemoimmunotherapy with hyper-CVAD plus rituximab for the treatment of adult Burkitt and Burkitt-type lymphoma or acute lymphoblastic leukemia. Cancer. 2006 ; 106i7j : 1569-80. i3iiiDj

5j Ottmann OG, et al ; GMALL Study Group. Imatinib compared with chemotherapy as front-line treatment of elderly patients with Philadelphia chromosome-positive acute lymphoblastic leukemia iPh{ALLj. Cancer. 2007 ; 109i10j : 2068-76.i1iiDivj

6j Delannoy A, et al. Imatinib and methylprednisolone alternated with chemotherapy improve the outcome of elderly patients with Philadelphia-positive acute lymphoblastic leukemia : results of the GRAALL AFR09 study. Leukemia. 2006 ; 20i9j : 1526-32.i3iiiDivj

7j Vignetti M, et al. Imatinib plus steroids induces complete remissions and prolonged survival in elderly Philadelphia chromosome-positive patients with acute lymphoblastic leukemia without additional chemotherapy : results of the Gruppo Italiano Malattie Ematologiche dellfAdultoiGIMEMAj LAL0201-B protocol. Blood. 2007 ; 109i9j : 3676-8.i3iiiDivj


CQ5

ˆź”Ź¬lPh ‰A«ALL ‚ĢŽ”—Ć–@‚Ķ‰½‚ŖŠ©‚ß‚ē‚ź‚é‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2B
Ph ‰A«ALL ‚É‘Ī‚·‚é•W€Ž”—Ć‚ĶŠm—§‚µ‚Ä‚¢‚Č‚¢B

y‰š ąz

30 ĪˆČć‚ĢPh ‰A«ALL ‚É‘Ī‚·‚鉻Šw—Ć–@‚Ģ¬Ń‚¾‚Æ‚š‚Ü‚Ę‚ß‚½˜_•¶‚ĶŒ©“–‚½‚ē‚Č‚¢‚ŖC2000 ”NˆČ~‚É”­•\‚³‚ź‚½200 —įˆČć‚Ģ¬lALL ‚ĢŽ”—Ɛ¬Ń‚š•\1 ‚ÉŽ¦‚·BŠ®‘SŠ°‰šiCRj—¦‚Ķ74`92“C‘S—į‚Ģ¶‘¶Š„‡‚Ķ27`54“‚Å‚ ‚éB¶‘¶Š„‡‚ÉŠÖ‚µ‚Ä‚ĶC—\ŒćˆöŽq‚šl—¶‚µ‚½‘¢ŒŒŠ²×–EˆŚAistem cell transplantationFSCTj‚Ŗ‰½‚ē‚©‚ĢŒ`‚ÅŠÜ‚Ü‚ź‚Ä‚Ø‚čC‰»Šw—Ć–@‚ĘˆŚA‚šƒpƒbƒP[ƒW‚Ę‚µ‚½Ž”—Ć–@‚Ģ¬Ń‚Ę—‰š‚·‚ׂ«‚Å‚ ‚éB

•\1@‘å‹K–Ķ¬lALL Ž”—ĆŒ¤‹†‚Ģ¬Ń
ŽŽŒ±–¼ ”­•\”N Œ¤‹†ŠśŠŌ Ē—į” ”N—ī
’†‰›’l
irangej
SCT Ph L3 CR —¦ ¶‘¶Š„‡
LALA 87 2000 Nov 1986`
July 1991
572 33
i15`60j
yes yes no 76“ 27“i10 ”Nj
GMALL 05/93– 2001 Apr 1993`
Oct 1999
1,163 35
i15`65j
yes yes no 83“ 35“i5 ”Nj
JALSG ALL93 2002 Dec 1993`
Feb 1997
263 31
i15`59j
yes yes yes 78“ 33“i6 ”Nj
GIMEMA 0288 2002 Jan 1988`
Apr 1996
778 27.5
i12`60j
yes yes no 82“ 27“i9 ”Nj
Hyper-CVAD 2004 Feb 1992`
Mar 2000
288 40
i15`92j
yes yes yes 92“ 38“i5 ”Nj
LALA 94 2004 Jun 1994`
Jan 2002
922 33
i15`55j
yes yes no 84“ 33“i5 ”Nj
MRC UKALL XII/
ECOG E2993
2005 1993`
2003
1,521 15`59 yes yes yes 91“ 38“i5 ”Nj
GMALL 07/2003– 2007 Apr 2003`
Dec 2006
713 34
i15`55j
yes yes no 89“ 54“i5 ”Nj
SWOG 9400 2008 Aug 1995`
May 2000
200 15`65 yes yes no 80“ 33“i5 ”Nj
JALSG ALL97 2010 May 1997
`Dec 2001
404 38
i15`64j
yes yes no 74“ 32“i5 ”Nj

–“˜^
SCTF‘¢ŒŒŠ²×–EˆŚA
PhFPh —z«—į
L3FFAB •Ŗ—ŽL3
yes ‚Ķć‹LĒ—į‚šŠÜ‚ŽCno ‚Ķć‹LĒ—į‚ĶœŠOB

ć‹L‚Ģ¬Ń‚Ģ‚¤‚æCPh ‰A«—į‚ ‚é‚¢‚Ķ”N—ī‚šƒTƒuƒOƒ‹[ƒv‚Ę‚µ‚Ä’Šo‚µ‚½Œ‹‰Ź‚šˆČ‰ŗ‚ÉŽ¦‚·BJapan Adult Leukemia Study GroupiJALSGjALL93 ‚É‚Ø‚Æ‚éPh ‰A«—į‚ĢCR —¦C¶‘¶Š„‡‚Ķ‚»‚ź‚¼‚ź83 “C39 “ ‚ŁC30 ĪˆČć‚ĢĒ—į‚Å‚ĶC72 “C21 “ ‚Å‚ ‚Į‚½1jBhyper-CVAD —Ć–@iCPA, VCR, DXR, DEXj‚Å‚ĢPh —z«Cƒo[ƒhƒLƒbƒgƒ^ƒCƒvˆČŠO‚ĢĒ—į‚Å‚ĶC‚»‚ź‚¼‚ź91“C41“‚Å‚ ‚čC40`59 Ī‚Å‚ĶC80“C30“‚Å‚ ‚Į‚½2jBMedical Research CounciliMRCj/Eastern Cooperative Oncology GroupiECOGj‚Å‚ĢPh ‰A«—į‚Å‚ĶC93“C44“‚Å‚ ‚čC35 ĪˆČć‚ĢCR —¦‚Ķ89“‚ŁC¶‘¶Š„‡‚Ķ30`39 ĪC40`49 ĪC50 ĪˆČć‚Å‚»‚ź‚¼‚ź34“C23“C15“‚Å‚ ‚Į‚½3jBSouthwestern Oncology GroupiSWOGj 9400 ‚É‚Ø‚Æ‚é30`49 ĪC50`65 Ī‚ĢCR —¦C¶‘¶Š„‡‚Ķ‚»‚ź‚¼‚ź80“C32“‚Ę63“C23“‚Å‚ ‚Į‚½4jBJALSG ALL97 ‚Å‚ĶCPh ‰A«ŒQ‚šƒTƒuƒOƒ‹[ƒv‚Ę‚µ‚ĉšĶ‚µCCR —¦81“C¶‘¶Š„‡39“‚Å‚ ‚Į‚½BPh ‰A«ŒQ‚Ģ”N—ī•Ź‚Ģ¬Ń‚ĶC35`54 ĪC55`64 Ī‚Å‚»‚ź‚¼‚ź80“C38“‚Ę78“C26“‚Å‚ ‚Į‚½5jB

ˆČć‚©‚ēCPh ‰A«ŒQ‚Ģ¶‘¶Š„‡‚Ķ‘SĒ—į‚šŠÜ‚Ž¶‘¶Š„‡‚ę‚č‚ā‚ā—ĒD‚ŁC30 ĪˆČć‚ĢĒ—į‚Ģ¶‘¶Š„‡‚Ķ‘S‘Ģ‚ę‚č‚ā‚ā•s—Ē‚Å‚ ‚邱‚Ę‚ĶŽ¦‚³‚ź‚½‚ŖCˆŁ‚Č‚éƒvƒƒgƒR[ƒ‹‚šƒ‰ƒ“ƒ_ƒ€‰»‚µ‚Ä”äŠr‚µ‚½ŽŽŒ±‚Ķ‚Č‚­CƒvƒƒgƒR[ƒ‹ŠŌ‚Ģ—D—ņ‚Ķ•s–¾‚Å‚ ‚éB

yŽQl•¶Œ£z

1j Takeuchi J et al. Induction therapy by frequent administration of doxorubicin with four other drugs, followed by intensive consolidation and maintenance therapy for adult acute lymphoblastic leukemia : the JALSG-ALL93 study. Leukemia. 2002 ; 16i7j : 1259-66.i3iiAj

2j Kantarjian H et al. Long-term follow-up results of hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone iHyper-CVADj, a dose-intensive regimen, in adult acute lymphoblastic leukemia. Cancer. 2004 ; 101i12j : 2788-801.i3iDiij

3j Rowe JM et al. Induction therapy for adults with acute lymphoblastic leukemia : results of more than 1500 patients from the international ALL trial : MRC UKALL XII/ECOG E2993. Blood. 2005 : 106i12j : 3760-7.i3iiAj

4j Pullarkat V et al. Impact of cytogenetis on the outcome of adult acute lymphoblastic leukemia : results of Southwest Oncology Group 6400 study. Blood. 2008 ; 111i5j : 2563-72.i3iDiij

5j Jinnai I et al. Intensifi ed consolidation therapy with dose-escalated doxorubicin did not improve the prognosis of adults with acute lymphoblastic leukemia : the JALSG-ALL97 study. Int J Hematol. 2010 ; 92i3j: 490-502.i3iDiij


CQ6

T ×–E«ALL ‚ĘB ×–E«ALL ‚Ķ“Æ‚¶Ž”—Ć•ū–@‚Å‚ę‚¢‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2B
‰ß‹Ž‚Ģ—Տ°ŽŽŒ±‚Ģ¬Ń‚©‚ēlŽ@‚·‚éŒĄ‚č‚Å‚ĶCT ×–E«ALL ‚ĘB ×–E« ALL ‚ÅŽ”—Ć‚š•Ļ‚¦‚é‚ׂ«‚Å‚ ‚é‚Ę‚¢‚¤–¾Šm‚ȍŖ‹’‚Ķ‘¶Ż‚µ‚Č‚¢B

y‰š ąz

T ×–E«ALLiT-ALLj‚Ķ‘SALL ‚Ģ20“‘OŒć‚É‚·‚¬‚øC‰ß‹Ž‚Ģ—Տ°ŽŽŒ±‚Å‚ĶB ×–E«ALLiB-ALLj‚ĘT ×–E«ALL ‚Ģ—¼ŽŅ‚šŠÜ‚ß‚Ä“Æ‚¶‰»Šw—Ć–@‚Ŗs‚ķ‚ź‚Ä‚«‚½i‚½‚¾‚µC‚ƒŠƒXƒNŒQ‚š’č‹`‚·‚éŪ‚Ģ‰fŽž”’ŒŒ‹…”‚É‚Ā‚¢‚Ä‚ĶT-ALL ‚Å‚ę‚č‚‚¢č‡’l‚šĢ—p‚µ‚Ä‚¢‚é‚ą‚Ģ‚Ŗ‘½‚¢jB‹ß”N‚Ģ‘å‹K–Ķ—Տ°ŽŽŒ±‚ĢŒ‹‰Ź‚š‚Ż‚é‚ĘT-ALL ‚ĢŽ”—Ɛ¬Ń‚ŖB-ALL ‚ę‚č‚ąŽįŠ±—D‚ź‚Ä‚¢‚é‚Ę‚¢‚¤‚ą‚Ģ‚Ŗ‘½‚¢‚ŖC‚»‚ĢŒ‹‰Ź‚Ķˆź—l‚Å‚Ķ‚Č‚¢BŠeŒ¤‹†ŠŌ‚ʼn»Šw—Ć–@‚Ģ“ą—e‚ą‘å‚«‚­ˆŁ‚Č‚éB

2000 ”NˆČ~‚É”­•\‚³‚ź‚½Ē—į”100 —įˆČć‚Ģ—Տ°ŽŽŒ±‚ĢŒ‹‰Ź‚šˆź——‚·‚é‚ʁC—į‚¦‚ĪÅ‚ą‹K–Ķ‚Ģ‘å‚«‚¢MRC UKALL XII/ECOG E2993 ‚Å‚Ķ5 ”N‘S¶‘¶Š„‡iOSj‚ŖT-ALL ‚Å48“CB-ALL ‚Å41“‚ĘT-ALL ‚Ŗ—LˆÓ‚É—D‚ź‚Ä‚¢‚½1jB‚±‚ĢŒ¤‹†‚Å‚Ķ‘å—ŹƒVƒ^ƒ‰ƒrƒ“iAraCj‚ĶŠÜ‚Ü‚ź‚Ä‚¢‚Č‚¢‚ŖC‘å—ŹƒƒgƒgƒŒƒLƒT[ƒgiMTXji3 g/m2j‚Ŗ3 ƒR[ƒX“Š—^‚³‚ź‚Ä‚¢‚éBMD ƒAƒ“ƒ_[ƒ\ƒ“‚Ŗ‚ńƒZƒ“ƒ^[‚Ģhyper-CVAD/MA —Ć–@‚ĶMTX ‚Ķ1 g/m2 ‚Ŗ‡Œv4 ‰ńCAraC ‚Ķ3 g/m2 ‚Ŗ‡Œv16 ‰ń“Š—^‚³‚ź‚éƒvƒƒgƒR[ƒ‹‚Å‚ ‚é2jBT-ALL ‚Ģ5 ”NOS ‚Ķ48“‚ŁC‚»‚źˆČŠO‚ĢALLiT-CALLAprecursor B-cell ALLCNULL ALL ‚šŠÜ‚Žj‚Ģ5 ”NOS 36“‚š—LˆÓ‚ɏć‰ń‚Į‚½B’†“™—Ź‚ĢMTXi600 mg/m2 ‚š‡Œv4 ‰ńj‚šĢ—p‚µ‚½“ś–{‚ĢJALSG-ALL93 ŽŽŒ±‚Å‚Ķ6 ”NOS ‚ŖT-ALL ‚Å41.5“CB-ALL ‚Å35.9“‚ĘT-ALL ‚Ŗ—D‚ź‚Ä‚¢‚é‚ą‚Ģ‚Ģ—LˆÓ·‚É‚ĶŽŠ‚Į‚Ä‚¢‚Č‚¢3jBˆź•ūC2002 ”N‚É”­•\‚³‚ź‚½GIMEMA ALL 0288 ŽŽŒ±‚Å‚ĶŠ®‘SŠ°‰š“ž’BŠ³ŽŅ‚Ģ8 ”NŒćŠ°‰šˆŪŽ—¦‚ĶB-ALL ‚Å34“CT-ALL ‚Å27“‚ĘB-ALL ‚Ŗ—LˆÓ‚É—D‚ź‚é‚Ę‚¢‚¤Œ‹‰Ź‚Å‚ ‚Į‚½4jB‚±‚ĢŒ¤‹†‚Å‚Ķ‘å—ŹAraC ‚Ķ—p‚¢‚ē‚ź‚Ä‚¢‚Č‚¢‚ŖC‹­‰»—Ć–@‚Å1 g/m2 ‚ĢMTX ‚Ŗ3 ƒR[ƒX“Š—^‚³‚ź‚Ä‚¢‚éB

‚±‚ź‚ē‚ĢŒ‹‰Ź‚š‘‡“I‚ɍl‚¦‚é‚ʁC‘å—ŹMTX ‚ĢĢ—p‚ĶT-ALL ‚É‚Ø‚¢‚Ä–£—Ķ“I‚Č‘I‘šŽˆ‚Å‚Ķ‚ ‚é‚ŖC•K‚ø‚µ‚ą‚»‚ź‚šŠÜ‚ŽŽŽŒ±‚É‚Ø‚¢‚ÄT-ALL ‚ĢŽ”—Ɛ¬Ń‚Ŗ—D‚ź‚Ä‚¢‚é‚Ę‚ĶŒ¾‚¦‚Č‚¢B”ŒćCƒlƒ‰ƒ‰ƒrƒ“iAraGj‚Č‚Ē‚Ģ–ņÜ‚š‘Šś‚©‚ē“±“ü‚·‚邱‚Ę‚É‚ę‚Į‚ÄT-ALL ‚ĢŽ”—Ɛ¬Ń‚ŖŒüć‚·‚é‰Ā”\«‚Ŗ‚ ‚é‚ŖCŒ»Žž“_‚Å‚Ķ—Տ°ƒf[ƒ^‚Ķ–R‚µ‚¢B

yŽQl•¶Œ£z

1j Rowe JM, et al. Induction therapy for adults with acute lymphoblastic leukemia : results of more than 1500 patients from the international ALL trial : MRC UKALL XII/ECOG E2993. Blood. 2005 ; 106i12j : 3760-7.i3iiAj

2j Kantarjian H, et al. Long-term follow-up results of hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone iHyper-CVADj, a dose-intensive regimen, in adult acute lymphocytic leukemia. Cancer. 2004 ; 101i12j : 2788-801.i3iiAj

3j Takeuchi J, et al. Induction therapy by frequent administration of doxorubicin with four other drugs, followed by intensive consolidation and maintenance therapy for adult acute lymphoblastic leukemia : the JALSG-ALL93 study. Leukemia. 2002 ; 16i7j : 1259-66.i3iiAj

4j Annino L, et al. Treatment of adult acute lymphoblastic leukemia iALLj : long-term follow-up of the GIMEMA ALL 0288 randomized study. Blood. 2002 ; 99i3j : 863-71.i1iiDii/3iiDiij


CQ7

¬lALL ‚ɂ؂Ƃ銰‰š“±“ü—Ć–@prephase ‚Å‚ĢƒvƒŒƒhƒjƒ]ƒƒ“”½‰ž«‚Ķ—\Œć”»’č‚É—L—p‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2B
¬lALL ‚ĢŠ°‰š“±“ü—Ć–@‚É‚Ø‚¢‚āCprephase ‚ĢPSL 7 “śŠŌ“Š—^‚ĢŒų‰Ź‚ŁC—\Œć‚š”»’f‚Å‚«‚é‰Ā”\«‚Ķ‚ ‚éB

y‰š ąz

¬Ž™‚ĢALL Ž”—Ć‚Å‚ĶC7 “śŠŌ‚ĢƒvƒŒƒhƒjƒ]ƒƒ“iPSLj“Š—^‚Ŗprephase ‚Ę‚µ‚čs‚ķ‚źCday 8 ‚Ģ––½ŒŒ’†‚Ģ‰č‹…‚Ŗ1,000/ƒŹl ˆČć‚ ‚ź‚Ī—\Œć•s—Ē‚Ę•ń‚³‚ź‚Ä‚¢‚é1jB‘½‚­‚Ģ¬Ž™ƒvƒƒgƒR[ƒ‹‚Å‚ĶC‚±‚ĢPSL ”½‰ž«‚É‚ę‚čƒŠƒXƒN‚šŒˆ’肵CŽ”—Ć–@‚š•Ļ‚¦‚Ä‚¢‚éB

prephase ‚ĢPSL “Š—^‚ĢŒų‰Ź‚šŒŸŲ‚µ‚½¬lƒvƒƒgƒR[ƒ‹‚ĶCGruppo Italiano Malattie EMatologiche dellf AdultoiGIMEMAjALL0288 ŽŽŒ±‚¾‚Æ‚Å‚ ‚é2jB‚±‚ĢŽŽŒ±‚Å‚ĶC7 “śŠŌ‚ĢPSL “Š—^i‘“Š—^—Ź330 mg/m2j‚Ŗs‚ķ‚źCprephase ˆČ‘O‚ÉPSL ‚š“Š—^‚³‚ź‚Ä‚¢‚½121 —į‚šœ‚­657 —į’†429 —įi69“j‚ÉŒų‰Ź‚Ŗ”F‚ß‚ē‚ź‚½B”’ŒŒ‹…”‚Ŗ‘½‚¢Ē—į‚Ø‚ę‚ŃB ×–E«ALLiB-ALLj‚É—LŒų—¦‚Ŗ‚‚©‚Į‚½B•]‰æ‰Ā”\769 —į‚ĢCR —¦‚Ķ82“‚ŁCPSL —LŒų—į‚ĢCR —¦‚Ķ”ń—LŒų—į‚ę‚č—D‚Į‚Ä‚¢‚½i87“ vs 70“Cp0.001jB‘SCR —į‚ĢCR Ž‘±Š„‡iCCRj‚Ķ33“i9 ”Nj‚ŁCPSL —LŒų—į‚Ģ8 ”NCCR ‚Ķ”ń—LŒų—į‚ę‚č—D‚Į‚Ä‚¢‚½i36“ vs 24“Cp0.0004jB

prephase ‚ĢPSL “Š—^‚šs‚¢C‚»‚Ģ”½‰ž«‚Ŗˆ«‚¢Ē—į‚ɁCŠ°‰š“±“ü—Ć–@‚ÅCY ‚š’Ē‰Į“Š—^‚·‚éGRAALL-2003 ŽŽŒ±‚Ģ¬Ń‚ĶCCR —¦93.5“i210/225jC‘S—į‚Ģ¶‘¶Š„‡60“i42 ƒJŒŽj‚Ę—ĒD‚Å‚ ‚Į‚½3jB‚µ‚©‚µC‚±‚Ģ—ĒD‚Ȑ¬Ń‚ŖCprephase ‚ĢPSL “Š—^‚É‚ę‚邽‚ß‚©‚Ē‚¤‚©‚Ķ–¾‚ē‚©‚Å‚Ķ‚Č‚¢B

yŽQl•¶Œ£z

1j Donadieu J, Hill C. Early response to chemotherapy as a prognostic factor in childhood acute lymphoblastic leukemia : a methodological review. Brit J Haematol. 2001 : 115i1j ; 34-45.i3iiiDivj

2j Annino L, et al. Treatment of adult acute lymphoblastic leukemia iALLj : long-term follow-up of the GIMEMA ALL 0288 randomized study. Blood. 2002 ; 99i3j : 863-71.i1iiDiij

3j Huguet F et al : Pediatric-inspired therapy in adults with Philadelphia chromosome-negative acute lymphoblastic leukemia : the GRAALL-2003 study. J Clin Oncol. 2009 ; 27i6j : 911-8.i3iiAj


CQ8

¬lALL ‚ĢŽ”—Ć‚É‚Ø‚¢‚Ä’†•_ŒoŒnÄ”­—\–h‚ĶŠ©‚ß‚ē‚ź‚é‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2B
‚·‚ׂĂĢĒ—į‚É‘Ī‚µ‚āCR‚Ŗ‚ńÜ‚Ģ‘o“ą“Š—^‚Ø‚ę‚Ń’†•_ŒoŒn‚Ö‚ĢˆŚs«‚Ģ—Ē‚¢‘Sg‰»Šw—Ć–@‚ĢŽg—p‚Ŗ„§‚³‚ź‚éB‘S”]ĘŽĖ‚Ķƒ‹[ƒ`ƒ“‚ÉŽ{s‚·‚ׂ«‚Å‚Ķ‚Č‚­C’†•_ŒoÄ”­ƒnƒCƒŠƒXƒN—į‚É‚Ø‚¢‚Ä‚Ķ‘I‘šŽˆ‚Ģˆź‚Ā‚Å‚ ‚éB

y‰š ąz

’†•_ŒoŒnicentral nervous systemFCNSjÄ”­‚Ö‚Ģ‘Īō‚ĶALL Ž”—Ɛ¬Ń‚ĢŒüć‚É•s‰ĀŒ‡‚Å‚ ‚éB‘Īō‚Ę‚µ‚āCR‚Ŗ‚ńÜ‚Ģ‘o“ą“Š—^iIntrathecalFITjC’†•_ŒoŒn‚Ö‚ĢˆŚs«‚Ģ—Ē‚¢‘Sg‰»Šw—Ć–@m‘å—ŹƒƒgƒgƒŒƒLƒT[ƒgiMTXjC‘å—ŹƒVƒ^ƒ‰ƒrƒ“iAraCjn‚Ø‚ę‚Ń‘S”]ĘŽĖ‚Ģ‘I‘šŽˆ‚Ŗ‚ ‚é1j`5jB‚±‚ź‚ē‚Ģ‘g‚Ż‡‚ķ‚¹‚Ø‚ę‚ŃŽ”—Ć‹­“x‚ĶCCNS Ä”­ƒŠƒXƒN‚ɉž‚¶‚Ä‘Īō‚šu‚¶‚é‚Ģ‚Ŗ–]‚Ü‚µ‚¢B

¬Ž™ALL ‚É”äŠr‚·‚é‚ʁC¬lALL ‚É‚Ø‚Æ‚éƒGƒrƒfƒ“ƒX‚Ķ‹É‚ß‚ÄŒĄ‚ē‚ź‚Ä‚¢‚āC•W€“I‘Īō–@‚ĶŠm—§‚³‚ź‚Ä‚¢‚Č‚¢B¬lALL Ž”—Ć‚É‚Ø‚Æ‚éCNS Ä”­—\–h–@‚ĢŠm—§‚Ģ‚½‚ß‚É‚ĶCˆČ‰ŗ‚Ģ3 “_‚Ŗ“Į‚ɏd—v‚ʍl‚¦‚ē‚ź‚éB

  1. CNS Ä”­ƒnƒCƒŠƒXƒNŒQ‚Ģ“Æ’č
  2. triple It (MTX{AraC{ƒXƒeƒƒCƒh‚Ģ3 Ü•¹—p)C‘å—ŹMTX ‚Ø‚ę‚Ń‘å—ŹAraC ‚Ģ“Š—^—ŹC“Š—^‰ń”‚ĢƒŠƒXƒN•ŹŽŠ“K‰»
  3. ƒnƒCƒŠƒXƒNŒQ‚É‚Ø‚Æ‚é‘S”]ĘŽĖ‚ĢˆÓ‹`

ˆČ‰ŗ‚ɁCŽå‚ɏ¬Ž™ALL ‚š‘ĪŪ‚Ę‚µ‚½—Տ°ŽŽŒ±‚É‚ę‚éƒGƒrƒfƒ“ƒX‚š—ń‹“‚µCŽQl‚Ę‚µ‚½‚¢B

MTX ’P“Ę“Š—^‚É”äŠr‚µ‚āCtriple IT ‚ŖCNS ’P“ĘÄ”­ƒŠƒXƒN‚š—LˆÓ‚É’į‰ŗip0.004j‚³‚¹‚é‚ŖCœ‘Ä”­‚Ķtriple IT ‚Å—LˆÓ‚É‘½‚©‚Į‚½ip0.01j6jB

‘S”]ĘŽĖ‚šŽ{s‚µ‚Č‚©‚Į‚½ź‡C5 ”NŠŌ‚Ģ—ŻĻCNS ’P“ĘÄ”­‚Ķ2.7“i95“ CIF1.1-4.3j‚Å‚ ‚čCf’fŽž CNS •a•Ļ‚šœ‚­CCNS Ä”­ƒŠƒXƒNˆöŽq‚Ķt (1;19)CT ×–E«‚Å‚ ‚Į‚½ 7jB

CNS Ä”­ƒŠƒXƒNˆöŽq‚Ķ•ń‚É‚ę‚Į‚ĈŁ‚Č‚é‚ŖCˆź”Ź‚ÉT ×–E«C‰fŽžWBC ‚’lC—\Œć•s—ĒõF‘ĢˆŁķ‚Ø‚ę‚ŃŠ°‰š“±“ü•s‰ž‚Č‚Ē‚Ŗ‚ ‚čC—\–h“I‘S”]ĘŽĖ‚Ģ‘ĪŪ‚Ę‚µ‚Ä‚¢‚éƒvƒƒgƒR[ƒ‹‚Ŗ‘½‚¢8jB

‘S”]ĘŽĖ‚É‚ę‚é”ÓŠś“Ő«‚Ę‚µ‚āC”’Žæ”]ĒC”F’m‹@”\’į‰ŗC“ą•Ŗ”åˆŁķ‚Ø‚ę‚ѐ‘–ŒŽī‚Č‚Ē‚Ģ“ńŽŸ«Žīį‡‚Č‚Ē‚Ŗ‚ ‚čC‘I‘š‚É‚Ķ’ˆÓ‚Ŗ•K—v‚Å‚ ‚é9j10jB

yŽQl•¶Œ£z

1j Larson RA, et al. A randomized controlled trial of filgrastim during remission induction and consolidation chemotherapy for adults with acute lymphoblastic leukemia : CALGB study 9111. Blood. 1998 ; 92 i5j : 1556-64.i3iDivj

2j Stock W, et al. What determines the outcomes for adolescents and young adults with acute lymphoblastic leukemia treated on cooperative group protocols? A comparison of Childrenfs Cancer Group and Cancer and Leukemia Group B studies. Blood. 2008 ; 112i5j : 1646-54.i3iiiAj

3j Kantarjian HM, et al. Results of treatment with hyper-CVAD, a dose-intensive regimen, in adult acute lymphocytic leukemia. J Clin Oncol. 2000 ; 18i3j : 547-61.i3iDivj

4j Huguet F, et al. Pediatric-inspired therapy in adults with Philadelphia chromosome-negative acute lymphoblastic leukemia : the GRAALL-2003 study. J Clin Oncol. 2009 ; 27i6j : 911-8.i3iiAj

5j Takeuchi J, et al. Induction therapy by frequent administration of doxorubicin with four other drugs, followed by intensive consolidation and maintenance therapy for adult acute lymphoblastic leukemia : the JALSG-ALL93 study. Leukemia. 2002 ; 16i7j : 1259-66.i3iiAj

6j Matloub Y, et al. Intrathecal triple therapy decreases central nervous system relapse but fails to improve event-free survival when compared with intrathecal methotrexate : results of the Childrenfs Cancer GroupiCCGj 1952 study for standard-risk acute lymphoblastic leukemia, reported by the Childrenfs Oncology Group. Blood. 2006 ; 108i4j : 1165-73.i1iiDivj

7j Pui CH, et al. Treating childhood acute lymphoblastic leukemia without cranial irradiation. N Engl J Med. 2009 ; 360i26j : 2730-41.i2Divj

8j Pui CH, et al. Current management and challenges of malignant disease in the CNS in paediatric leukaemia. Lancet Oncol. 2008 ; 9i3j : 257-68.i3iiiDj

9j Filley CM, et al. Toxic leukoencephalopathy. N Engl J Med. 2001 ; 345i6j : 425-32.i3iiiDivj

10j Hijiya N, et al. Cumulative incidence of secondary neoplasms as a first event after childhood acute lymphoblastic leukemia. JAMA. 2007 ; 297i11j : 1207-15.i3iiiDivj


CQ9

Š°‰šŠś¬lALL ‚ĢŽ”—Ć‚É‚Ø‚Æ‚é”÷¬Žc‘¶•a•Ļ‚Ģ•]‰æ‚ĢˆÓ‹`‚Ķ‚ ‚é‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2A
Š°‰š—Ć–@Œć‚É”÷¬Žc‘¶•a•Ļ‚Ŗ‚ ‚ź‚Īi0.1“‚Č‚¢‚µ0.01“ˆČćjCÄ”­‚ĢŠėŒÆ«‚Ŗ‚‚Ü‚éBŠ°‰š—Ć–@Œć‚Ģ‚Ē‚ĢŽž“_‚Å”÷¬Žc‘¶•a•Ļ‚š‘Ŗ’č‚·‚ׂ«‚©‚ĢƒRƒ“ƒZƒ“ƒTƒX‚Ķ‚Č‚¢B

y‰š ąz

”÷¬Žc‘¶•a•Ļiminimal residual diseaseFMRDj‚š‚Ż‚邽‚ß‚É‚ĶCALL ‚Ģ‰fŽž‚ɖʉuƒOƒƒuƒŠƒ“iIgj‚Č‚¢‚µT ×–EŽó—e‘ĢiTCRjˆā“`ŽqÄ\¬‚šŒ©o‚µC‚±‚ĢÄ\¬‚ĢƒAƒŠƒ‹“ĮˆŁ“Iƒvƒ‰ƒCƒ}[‚šģ¬‚µ‚Ä‚Ø‚­•K—v‚Ŗ‚ ‚éBŽ”—ĆŒć‚ĢŒŸ‘Ģ’†‚É“Æ‚¶”z—ń‚Ŗ‚ ‚é‚©‚Ē‚¤‚©‚šPCRipolymerase chain reactionj–@‚Å“Æ’č‚·‚éB‚Ü‚½CBCR-ABL ‚Č‚Ē‚ĢƒLƒƒ‰ˆā“`Žq‚āˆŁķ‚Č•\–Źƒ}[ƒJ[iaberrant markerj‚š‰fŽž‚ÉŒŸø‚µC‚±‚ź‚ē‚ĢˆŁķ‚šMRD ‘Ŗ’č‚É—p‚¢‚邱‚Ę‚Ŗ‚Å‚«‚é‚ŖCƒLƒƒ‰ˆā“`ŽqŒŸø‚Ģ•ŪŒÆ“K—p‚Ķˆź•”‚Ģ•a‰@‚ÉŒĄ‚ē‚ź‚Ä‚¢‚éB

Ph ‰A«ALL 116 —į‚š‘ĪŪ‚Ę‚µ‚āCaberrant marker ‚šMRD ‚ĢŽw•W‚Ę‚µ‚½Œć•ūŽ‹“IŒ¤‹†‚Å‚ĶCŠ°‰š“±“üI—¹Žž‚ĢMRD ‚Ķ“Ę—§‚µ‚½Ä”­‚Ģ—\ŒćˆöŽq‚Å‚ ‚Į‚½‚Ŗipƒ0.0001jC’nŒÅ‚ߌć‚ĢMRD ‚Ķ—\Œć‚Ę—LˆÓ‚ČŠÖ˜A‚Ķ‚Č‚©‚Į‚½1jBˆź•ūCT ×–EŒnˆČŠO‚ĢPh ‰A«ALL 161 —į‚š‘ĪŪ‚Ę‚µ‚½Œć•ūŽ‹“IŒ¤‹†‚Å‚ĶCŽ”—ĆŠJŽn1 ”NˆČ“ą‚Ģ‚Ē‚ĢŽž“_‚Å‚ąMRD ‚Ŗ—z«‚Å‚ ‚ź‚Ī—LˆÓ‚ɍĔ­‚Ü‚Å‚ĢŠśŠŌ‚Ŗ’Z‚©‚Į‚½2jB

–¢Ž”—Ć‚ĢB ×–E«ALL ‚Ø‚ę‚ŃT ×–E«ALL 142 —į‚š‘ĪŪ‚Ę‚µ‚½‘O•ūŽ‹“IŒ¤‹†‚Ŗs‚ķ‚ź‚½3jBŽ”—ĆŒć16 T‚Å0.01“–¢–ž‚Å‚ ‚čC22 T‚Å‘S‚­ŒŸo‚³‚ź‚Č‚¢‚ą‚Ģ‚šMRD ‰A«C‚»‚źˆČŠO‚šMRD —z«‚Ę’č‹`‚µC5 ”N‘S¶‘¶Š„‡iOSj/ –³Ä”­¶‘¶Š„‡iRFSj‚š”äŠr‚·‚é‚Ę—LˆÓ‚ÉMRD ‰A«ŒQ‚Ŗ—ĒD‚Å‚ ‚Į‚½i75“/72“ vs 33“/14“Cp0.001jB‚Ü‚½C196 —į‚Ģ•W€ƒŠƒXƒNALL ‚š‘ĪŪ‚Ę‚µ‚½‘O•ūŽ‹“IŒ¤‹†‚Å‚ĶCŠ°‰š“±“ü—Ć–@’†iday11j‚Ø‚ę‚ŃŠ°‰š“±“ü—Ć–@I—¹Žžiday24j‚ÉMRD ‰A«‚Å‚ ‚ź‚Ī3 ”NÄ”­—¦0“‚É‘Ī‚µCŽ”—ĆŒć16 T‚Ü‚ÅMRD —z«‚Å‚ ‚ź‚Ī3 ”NÄ”­—¦96“‚Ę•ń‚³‚ź‚Ä‚¢‚é4jB“ś–{‚Å‚ą¬lALL 27 —į‚š‘ĪŪ‚ɁCŠ°‰šI—¹Œć100 “ś‚ÉMRD ‚š‘Ŗ’肵‚½‘O•ūŽ‹“IŒ¤‹†‚Ŗ‚ ‚é5jBMRD ‰A«ŒQ‚Ģ2 ”NOS/RFS ‚Ķ79.0“/79.4“‚ŁC—z«ŒQ‚Ģ45.0“/40.0“‚ɔ䂵‚Ä—LˆÓ‚ɍ‚‚©‚Į‚½B

yŽQl•¶Œ£z

1j Holowiecki J, et al. Status of minimal residual disease after induction predicts outcome in both standard and high-risk Ph-negative adult acute lymphoblastic leukemia. The Polish Adult Leukemia Group ALL 4-2002 MRD study. Br J Haematol. 2008 ; 142i2j : 227-37.i3iDiij

2j Patel B, et al. Minimal residual disease is a signifi cant predictor of treatment failure in non T-lineage adult acute lymphoblastic leukaemia : final results of the international UKALL XII/ECOG E2993. Br J Haematol. 2009 ; 148i1j : 80-9.i3iDiij

3j Bassan R, et al. Improved risk classification for risk-specific therapy based on the molecular study of minimal residual disease iMRDj in adult acute lymphoblastic leukemia iALLj Blood. 2009 ; 113 i18j : 4153-62.i3iDiij

4j Bruüggemann M, et al. Clinical significance of minimal residual disease quantifi cation in adult patients with standard-risk acute lymphoblastic leukemia. Blood. 2006 ; 107i3j : 1116-23.i3iDiij

5j Kikuchi M, et al. Clinical significance of minimal residual disease in adult acute lymphoblastic leukemia. Int J Hematol. 2010 ; 92i3j : 481-9.i3iDiij


CQ10

¬lALL ‚ĢŠ°‰šŒć—Ć–@‚É‚Ø‚¢‚Ä‘å—ŹƒVƒ^ƒ‰ƒrƒ“‚ā‘å—ŹƒƒgƒgƒŒƒLƒT[ƒg‚ĶŠ©‚ß‚ē‚ź‚é‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2B
¬lALL ‚É‘Ī‚·‚銰‰šŒć—Ć–@‚Ę‚µ‚Ä‘å—ŹƒVƒ^ƒ‰ƒrƒ“i1`3 g/m2j‚ā‘å—ŹƒƒgƒgƒŒƒLƒT[ƒgi1`3 g/m2j‚Ķ‘Ć“–‚Č‘I‘šŽˆ‚Å‚ ‚éBÄ”­E“ļŽ”—į‚É‘Ī‚·‚é‹~‰‡‰»Šw—Ć–@‚Ę‚µ‚Ä‚ą—p‚¢‚ē‚ź‚éB

y‰š ąz

¬lALL ‚É‘Ī‚µ‚āC‘å—ŹƒVƒ^ƒ‰ƒrƒ“iAraCj—Ć–@‚ā‘å—ŹƒƒgƒgƒŒƒLƒT[ƒgiMTXj—Ć–@‚ĶC“–‰CŽå‚ɍĔ­E“ļŽ”—į‚É‘Ī‚·‚é‹~‰‡—Ć–@‚Ę‚µ‚Ä—p‚¢‚ē‚ź‚½B‘å—ŹMTX —Ć–@‚Ķ’P“ʁC‚ą‚µ‚­‚ĶƒAƒ“ƒgƒ‰ƒTƒCƒNƒŠƒ“ŒnRŽīį‡–ņ‚Ü‚½‚Ķƒ~ƒgƒLƒTƒ“ƒgƒƒ“iMITj‚Ę‚Ģ•¹—p—Ć–@‚Ę‚µ‚Ä—p‚¢‚ē‚ź‚é1j2jB‚»‚ĢŒćCALL ‚Ģ‰‰ńŽ”—Ć‚Å‚ą‘å—ŹAraC —Ć–@‚Ŗ—p‚¢‚ē‚ź‚é‚ꂤ‚É‚Č‚Į‚Ä‚«‚½BÅ‹ßC¬lALL ‚É‘Ī‚µ‚Ä‘å—ŹAraC —Ć–@C‘å—ŹMTXC‚ą‚µ‚­‚Ķ‚»‚Ģ—¼ŽŅ‚šŠ°‰šŒć—Ć–@‚Ę‚µ‚Ä—p‚¢‚½Ž”—ĆƒvƒƒgƒR[ƒ‹‚Ģ•ń‚Ŗ‘‚¦‚Ä‚Ø‚čC‚±‚ź‚ē‚Ģ“±“ü‚Ŗ¬lALL ‚Ģ—\Œć‰ü‘P‚ÉŠń—^‚µ‚Ä‚¢‚é‰Ā”\«‚Ŗ‚ ‚éB‚±‚ź‚ē‚ĢŽ”—Ć‚Å‚Ķ’†•_ŒoŒnÄ”­—\–hŒų‰Ź‚ąŠś‘Ņ‚³‚ź‚éB‚µ‚©‚µŒ»Ż‚܂łɐ¬lALL ‚É‚Ø‚¢‚Ä‘å—ŹAraCC‘å—ŹMTX ‚»‚ź‚¼‚ź‚ĢˆÓ‹`‚šŒŸŲ‚µ‚½ƒ‰ƒ“ƒ_ƒ€‰»”äŠrŽŽŒ±‚Ķ•ń‚³‚ź‚Ä‚Ø‚ē‚øCÅ“K‚Č—p—ŹE—p–@‚ąŠm—§‚µ‚Ä‚¢‚Č‚¢B

¬lALL ‚É‘Ī‚·‚銰‰šŒć—Ć–@‚É‘å—ŹAraCC‘å—ŹMTX ‚Ģ—¼ŽŅ‚š—p‚¢‚éƒvƒƒgƒR[ƒ‹‚Å‚ĶCMD ƒAƒ“ƒ_[ƒ\ƒ“‚Ŗ‚ńƒZƒ“ƒ^[‚©‚ē•ń‚³‚ź‚Ä‚¢‚éhyper-CVAD/MA —Ć–@3jiCPA, VCR, DXR, DEXC‚—p—ŹMTXC‚—p—ŹAraCj‚Ŗ‘ć•\“I‚Č‚ą‚Ģ‚Å‚ ‚éB‚±‚Ģ‘¼‚É‚ąPETHEMA ALL-93 ŽŽŒ±4jBGRAALL-2003 ŽŽŒ±5j‚Å‚ąŠ°‰šŒć—Ć–@‚É‘å—ŹAraCC‘å—ŹMTX ‚Ģ—¼•ū‚Ŗ—p‚¢‚ē‚ź‚½B‚Ü‚½MRC UKALL XII/ECOG E2993 ŽŽŒ±‚Å‚ĶŠ°‰šŒć—Ć–@‚É‘å—ŹMTX ‚Ŗ—p‚¢‚ē‚ź‚½6jB

“ś–{‘“ą‚ōs‚ķ‚ź‚½Ž”—ĆŒ¤‹†‚Ģ‚¤‚æJALSG ALL93 ŽŽŒ±‚āALL97 ŽŽŒ±‚Å‚ĶŠ°‰šŒć—Ć–@‚É’†“™—ŹMTXi500`600 mg/m2j‚Ŗ—p‚¢‚ē‚ź‚½BJALSG ALL 202-O ŽŽŒ±‚Å‚ĶŠ°‰šŒć—Ć–@‚Ģˆź•”‚Å‘å—ŹMTXi3 g/m2j‚Ę’†“™—ŹMTXi500 mg/m2j‚Ģ”äŠrŽŽŒ±‚Ŗs‚ķ‚ź‚Ä‚Ø‚čC¬lALL ‚ĢŠ°‰šŒć—Ć–@‚É‚Ø‚Æ‚éMTX ‚Ģ“KŲ‚Č—p—ŹE—p–@‚Ŗ–¾‚ē‚©‚ɂȂ邱‚Ę‚ŖŠś‘Ņ‚³‚ź‚éB

yŽQl•¶Œ£z

1j Kantarjian HM, et al. Mitoxantrone and high-dose cytosine arabinoside for the treatment of refractory acute lymphocytic leukemia. Cancer. 1990 ; 65i1j : 5-8.i3iiDiij

2j Camera A, et al. GIMEMA ALL-Rescue 97 : a salvage strategy for primary refractory or relapsed adult acute lymphoblastic leukemia. Haematologica. 2004 ; 89i2j : 145-53.i3iDivj

3j Kantarjian HM, et al. Results of treatment with hyper-CVAD, a dose-intensive regimen, in adult acute lymphocytic leukemia. J Clin Oncol. 2000 ; 18i3j : 547-61.i3iDivj

4j Ribera JM, et al. Comparison of intensive chemotherapy, allogeneic or autologous stem cell transplantation as post-remission treatment for adult patients with high-risk acute lymphoblastic leukemia. Results of the PETHEMA ALL-93 trial. Haematologica. 2005 ; 90i10j : 1346-56.i1iiDiij

5j Huguet F, et al. Pediatric-inspired therapy in adults with Philadelphia chromosome-negative acute lymphoblastic leukemia : the GRAALL-2003 study. J Clin Oncol. 2009 ; 27i6j : 911-8.i3iiAj

6j Rowe JM, et al. Induction therapy for adults with acute lymphoblastic leukemia : results of more than 1500 patients from the international ALL trial : MRC UKALL XII/ECOG E2993. Blood. 2005 ; 106i12j : 3760-7.i3iiAj


CQ11

¬lPh ‰A«ALL ‚É‘Ī‚·‚é’nŒÅ‚ß—Ć–@‚Ę‚µ‚ÄŽ©‰Ę‘¢ŒŒŠ²×–EˆŚA•¹—p‘å—Ź‰»Šw—Ć–@‚Ķ‘Ć“–‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[4
¬lPh ‰A«ALL ‚Ģ‰‰ńŠ°‰š—į‚É‘Ī‚·‚é‘å—Ź‰»Šw—Ć–@EŽ©‰Ę‘¢ŒŒŠ²×–EˆŚA‚Å‚ĶC‰»Šw—Ć–@‚Ģ‚Ż‚É‚ę‚銰‰šŒć—Ć–@‚Ę”ä‚ׂė\Œć‰ü‘P‚ĶŠś‘Ņ‚Å‚«‚øCs‚¤‚ׂ«‚Å‚Č‚¢B

y‰š ąz

¬lPh ‰A«ALL ‚É‚Ø‚¢‚āC]—ˆ‚Ģ‰»Šw—Ć–@‚Ģ‚Ż‚É‚ę‚銰‰šŒć—Ć–@‚Ę”äŠr‚µ‚ÄŽ©‰Ę‘¢ŒŒŠ²×–EˆŚA•¹—p‘å—Ź‰»Šw—Ć–@ihigh-dose chemotherapy with autologous hematopoietic stem cell transplantationFHDC/AHSCTj‚É‚ę‚é—\Œć‰ü‘P‚ŖŠś‘Ņ‚³‚ź‚½B‚µ‚©‚µCŽå‚ÉHLA ˆź’v“Æ–Eƒhƒi[‚Ŗ‚¢‚Č‚¢ALL Š³ŽŅ‚š‘ĪŪ‚Ę‚µ‚čs‚ķ‚ź‚½•””‚Ģƒ‰ƒ“ƒ_ƒ€‰»”äŠrŽŽŒ±‚É‚Ø‚¢‚ÄŽ©‰ĘˆŚAŒQ‚Ķ‰»Šw—Ć–@ŒQ‚Ę”äŠr‚µ‚Ä–³•a¶‘¶ŠśŠŌ‚ā‘S¶‘¶ŠśŠŌ‚É‚Ø‚Æ‚é—˜“_‚ĶŽ¦‚³‚ź‚Č‚©‚Į‚½B

ƒXƒyƒCƒ“‚ĢPETHEMA ƒOƒ‹[ƒv‚Ŗ‚ƒŠƒXƒNALL ‚š‘ĪŪ‚Ę‚µ‚čs‚Į‚½—Տ°ŽŽŒ±‚Å‚ĶCHLA ˆź’v“Æ–Eƒhƒi[‚Ŗ‚¢‚Č‚¢Š³ŽŅ‚šŠ°‰š“±“ü—Ć–@Œć‚ÉŽ©‰ĘˆŚAŒQC‰»Šw—Ć–@ŒQ‚É•Ŗ‚Æ‚½‚ŖC—¼ŒQ‚Ģ5 ”N–³•a¶‘¶Š„‡‚É—LˆÓ·‚Ķ”F‚ß‚ē‚ź‚Č‚©‚Į‚½1jBƒtƒ‰ƒ“ƒX‚ĢLALA-94 ŽŽŒ±‚Å‚ĶŠ°‰š“±“ü—Ć–@‚ÅŠ®‘SŠ°‰šiCRj‚ÉŽŠ‚Į‚½‰”­Žž‚ƒŠƒXƒNALL ‚ĢŠ³ŽŅ‚ʏ‰‰ńŠ°‰š“±“ü—Ć–@‚ÅŠ®‘SŠ°‰š‚ÉŽŠ‚ē‚ø‹~‰‡—Ć–@‚É‚ę‚芮‘SŠ°‰š‚Ŗ“¾‚ē‚ź‚½Š³ŽŅ‚šŽ©‰ĘˆŚAŒQ‚ʉ»Šw—Ć–@ŒQ‚É•Ŗ‚Æ”äŠr‚µ‚½‚Ę‚±‚ėC‰»Šw—Ć–@ŒQ‚ŔӊśÄ”­‚Ŗ‘½‚¢ŒXŒü‚Ŗ‚ ‚Į‚½‚ŖC–³•a¶‘¶ŠśŠŌ’†‰›’l‚É‚Ķ—LˆÓ·‚Ŗ‚Ż‚ē‚ź‚Č‚©‚Į‚½2jBMRC UKALL XII/ECOG E2993 ŽŽŒ±‚Å‚ĶC¬lALL ‚ÅŠ°‰š“±“üŒć‚ÉHLA ˆź’vŒŒ‰ƒhƒi[‚Ŗ‚¢‚Č‚¢Š³ŽŅ‚Ŗ‰»Šw—Ć–@ŒQ‚©Ž©‰ĘˆŚAŒQ‚Ģ‚¢‚ø‚ź‚©‚Ƀ‰ƒ“ƒ_ƒ€‰»Š„‚č•t‚Æ‚³‚ź‚½B5 ”N‘S¶‘¶Š„‡‚Ķ46“ vs 37“ip0.03j‚ŁC‚Ž‚µ‚ė‰»Šw—Ć–@ŒQ‚Ģ•ū‚Ŗ—ĒD‚ČŽ”—Ɛ¬Ń‚Ę‚Č‚Į‚½3jB

yŽQl•¶Œ£z

1j Ribera JM, et al. Comparison of intensive chemotherapy, allogeneic or autologous stem cell transplantation as post-remission treatment for adult patients with high-risk acute lymphoblastic leukemia. Results of the PETHEMA ALL-93 trial. Haematologica. 2005 ; 90i10j : 1346-56.i1iiDiij

2j Thomas X, et al. Outcome of treatment in adults with acute lymphoblastic leukemia : analysis of the LALA-94 trial. J Clin Oncol. 2004 ; 22i20j : 4075-86.i1iiDiij

3j Goldstone AH, et al. In adults with standard-risk acute lymphoblastic leukemia, the greatest benefit is achieved from a matched sibling allogeneic transplantation in first complete remission, and an autologous transplantation is less effective than conventional consolidation/maintenance chemotherapy in all patients : final results of the International ALL Trial iMRC UKALL XII/ECOG E2993j. Blood. 2008 ; 111 i4j : 1827-33.i1iiAj


CQ12

cŠu•a•Ļ‚š—L‚·‚éT ×–E«LBL ‚É‘Ī‚µ‚ďcŠuĘŽĖ‚Ķs‚¤‚ׂ«‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2B
cŠu•a•Ļ‚š—L‚·‚鐬lT ×–E«LBL ‚É‘Ī‚µ‚āC‹ĒŠÄ”­—\–h‚š–Ś“I‚Ę‚µ‚½cŠuĘŽĖ‚Ŗ—p‚¢‚ē‚ź‚邱‚Ę‚Ŗ‘½‚¢‚ŖC•Ä‘‚ĢCALGB 8811 ƒvƒƒgƒR[ƒ‹‚Č‚ĒcŠuĘŽĖ‚šŠÜ‚Ü‚Č‚¢ALL ‚É‘Ī‚·‚鎔—ĆƒvƒƒgƒR[ƒ‹‚š—p‚¢‚邱‚Ę‚ą‘Ć“–‚Å‚ ‚éB

y‰š ąz

T ×–E«LBLiT-LBLj‚Å‚Ķf’fŽž‚ɏcŠuŽīįŽ‚š—L‚·‚é—į‚Ŗ‘½‚­C‚±‚ź‚Ŗ’·Œa10 cm ‚š’“‚¦‚鋐‘å•a•Ļ‚Å‚ ‚邱‚Ę‚ą­‚Č‚­‚Č‚¢BcŠuŽīįŽ‚š—L‚·‚éT-LBL ‚Å‚ĶC‘S—įC‚ ‚é‚¢‚Ķ‹‘å•a•Ļ‚š—L‚·‚鏼‡‚ā‰»Šw—Ć–@Œć‚ÉŽc‘¶ŽīįŽ‚Ŗ”F‚ß‚ē‚ź‚½ź‡‚ÉŒĄ’肵‚Ä‹ĒŠÄ”­—\–h‚š–Ś“I‚Ę‚µ‚½cŠuĘŽĖ‚Ŗs‚ķ‚ź‚邱‚Ę‚Ŗ‘½‚¢B‚µ‚©‚µC‚±‚ź‚É‚ę‚é“ńŽŸ”­Šą‚āSŒŒŠĒŒn‡•¹Ē‚Č‚Ē‚Ģ”ÓŠśįŠQ‚Ģ‘‰Į‚ŖŒœ”O‚³‚ź‚Ä‚¢‚éB¬Ž™LBL ‚Å‚ĶCALL ‚É‘Ī‚·‚é‹­—Ķ‚ȉ»Šw—Ć–@‚šs‚¤‚±‚Ę‚É‚ę‚čCcŠuĘŽĖ‚šŠÜ‚ŽƒvƒƒgƒR[ƒ‹‚Ę”äŠr‚µ‚Ä‘»F‚Č‚¢Ž”—Ɛ¬Ń‚Ŗ•ń‚³‚ź‚Ä‚¢‚é1jBcŠu•a•Ļ‚š—L‚·‚鐬lLBL ‚Å‚ĢcŠuĘŽĖ‚ĢˆÓ‹`‚ĶC”‚Ģ‚Ę‚±‚ė–¢‰šŒˆ‚Ģ‰Ū‘č‚Å‚ ‚éB

¬lLBL ‚Å‚ĶŒć•ūŽ‹“IŒ¤‹†‚É‚Ø‚¢‚Ähyper-CVAD —Ć–@iCPA, VCR, DXR, DEXj‚š’†S‚Ę‚·‚鉻Šw—Ć–@‚šŽó‚Æ‚½Š³ŽŅ‚ŁCcŠuĘŽĖ—į‚Ŗ”ńĘŽĖ—į‚É”ä‚ׂċĒŠÄ”­—¦‚Ŗ’į‚¢‚±‚Ę‚ŖŽ¦‚³‚ź‚½2jB‚±‚ź‚šŽó‚Æ‚ÄLBL ‚š‘ĪŪ‚Ę‚µ‚½hyper-CVAD/MA —Ć–@iCPA, VCR, DXR, DEXC‚—p—ŹMTXC‚—p—ŹAraCj‚Å‚ĶCf’fŽž‚ɏcŠu•a•Ļ‚š—L‚µ‚½Š³ŽŅ‚Å’nŒÅ‚ß—Ć–@‚ĘˆŪŽ—Ć–@‚ĢŠŌ‚ɏcŠuĘŽĖi30`39 Gyj‚Ŗ‹K’č‚³‚ź‚½3jBƒhƒCƒc‚ĢBerlin-Frankfurt-MunsteriBFMjƒŒƒWƒƒ“‚Å‚ąCcŠu•a•Ļ‚š—L‚·‚éT-LBL ‚ÅŠ°‰š“±“ü—Ć–@‚ĢŒć”¼‚ɏcŠuĘŽĖi24 Gyj‚šs‚¤‚±‚Ę‚Ŗ‹K’č‚³‚ź‚½B‚µ‚©‚µÄ”­—į‚Ģ”¼”‚ŏcŠuÄ”­‚Ŗ‚Ż‚ē‚źC‚±‚ź‚ē‚Ģ‚Ł‚Ę‚ń‚Ę‚ÅcŠuĘŽĖ‚ĢŠł‰‚Ŗ‚ ‚Į‚½‚½‚߁C˜_•¶‚Å‚ĶC‚ę‚č‚—p—Ź‚ĢcŠuĘŽĖ‚Ŗ•K—v‚¾‚ė‚¤‚ĘŒ‹˜_‚µ‚Ä‚¢‚é4jB‚µ‚©‚µC‚±‚ź‚ē‚Ģ2 ‚Ā‚ĢƒvƒƒgƒR[ƒ‹‚ĶcŠuĘŽĖ‚Ģ—L–³‚šƒ‰ƒ“ƒ_ƒ€‰»‚µ‚ÄŒŸ“¢‚µ‚½‚ą‚Ģ‚Å‚Ķ‚Č‚¢B‘ĪŪŽ¾Š³‚š¬lLBL ‚ÉŒĄ’肵‚½CcŠuĘŽĖ‚šs‚ķ‚Č‚¢ƒvƒƒgƒR[ƒ‹‚š—p‚¢‚½Ž”—ĆŒ¤‹†‚Å‚Ķ–Ś—§‚Į‚½•ń‚Ŗ‚Č‚¢‚ŖC•Ä‘‚ĢCALGB 8811 ‚Ģ•ń‚Å‚ĶC‘ĪŪŠ³ŽŅ‚Ģ15“i30 lj‚ŖcŠu•a•Ļ‚š—L‚µ‚Ä‚Ø‚čCcŠuĘŽĖ‚šs‚ķ‚Č‚¢ƒvƒƒgƒR[ƒ‹‚Å‚ ‚Į‚½‚É‚ą‚©‚©‚ķ‚ē‚øCT-ALL Š³ŽŅ‚¾‚ƂʼnšĶ‚µ‚Ä‚ąCcŠu•a•Ļ‚š—L‚·‚邱‚Ę‚Ķ—\Œć—ĒDˆöŽq‚Å‚ ‚Į‚½5jB‚Ń‚Ü‚ń«‘å×–EŒ^B ×–EƒŠƒ“ƒpŽī‚Č‚Ē‘¼‚Ģ•aŒ^‚ĢƒŠƒ“ƒpŽī‚Ę“Æ—l‚ɁCcŠuĘŽĖ‚Ģ“K‰ž‚šCŠ°‰š“±“ü‰»Šw—Ć–@Œć‚ĢŽc‘¶ŽīįŽ‚āpositron emission tomographyiPETj—z«Žc‘¶•a•Ļ‚Č‚Ē‚ÉŒĄ’č‚·‚é‚Ę‚¢‚¤•ūj‚šŽxŽ‚·‚é—Տ°Œ¤‹†‚Ģ•ń‚Ķ‚Č‚¢B

yŽQl•¶Œ£z

1j Reiter A, et al. Intensive ALL-type therapy without local radiotherapy provides a 90“ event-free survival for children with T-cell lymphoblastic lymphoma : a BFM group report. Blood. 2000 ; 95i2j : 416-21.i3iiiDij

2j Dabaja BS, et al. The role of local radiation therapy for mediastinal disease in adults with T-cell lymphoblastic lymphoma. Cancer. 2002 ; 94i10j : 2738-44.i3iiiDiij

3j Thomas DA, et al. Outcome with the hyper-CVAD regimens in lymphoblastic lymphoma. Blood 2004 ; 104i6j : 1624-30i3iiiAj

4j Hoelzer D, et al. Outcome of adult patients with T-lymphoblastic lymphoma treated according to protocols for acute lymphoblastic leukemia. Blood. 2002 ; 99i12j : 4379-85.i3iiiAj

5j Larson RA, et al. A five-drug remission induction regimen with intensive consolidation for adults with acute lymphoblastic leukemia : cancer and leukemia group B study 8811. Blood. 1995 ; 85 i8j : 2025-37.i3iDivj


CQ13

‘ęˆźŠ°‰šŠś‚Ģ“ÆŽķ‘¢ŒŒŠ²×–EˆŚA‚Ķ‚Ē‚Ģ‚ꂤ‚ȏĒ—į‚É“K‰ž‚³‚ź‚é‚ׂ«‚©iPh —z«CPh ‰A«‚šŠÜ‚Žj


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[1
‘ęˆźŠ°‰šŠś‚Ģ¬lALL ‚É‘Ī‚µ‚āCHLA “K‡ŒŒ‰C”ńŒŒ‰ƒhƒi[‚Ŗ‚¢‚ź‚Ī“ÆŽķ‘¢ŒŒŠ²×–EˆŚA‚É‚ę‚Į‚摶Š„‡‚Ģ‰ü‘P‚ŖŠś‘Ņ‚Å‚«‚éB‚µ‚©‚µC”Œć‚Ģ‰»Šw—Ć–@‚Ģ‰ü‘P‚É‚ę‚Į‚ÄŒ‹˜_‚Ŗ•Ļ‰»‚·‚é‰Ā”\«‚Ŗ‚ ‚éB

y‰š ąz

‘ęˆźŠ°‰šŠśALL ‚É‘Ī‚·‚é‘¢ŒŒŠ²×–EˆŚA‚Ģ“K‰ž‚Ķgenetic randomizationC‚·‚Č‚ķ‚æ‘ęˆźŠ°‰š‚Ŗ“¾‚ē‚ź‚½Š³ŽŅ‚šHLA “K‡“Æ–Eƒhƒi[‚Ŗ‚¢‚鏼‡‚É‚Ķ“ÆŽķˆŚAŒQ‚ÉŠ„‚č•t‚ƁCƒhƒi[‚Ŗ‚¢‚Č‚¢ź‡‚É‚ĶŽ©‰Ęœ‘ˆŚAŒQ‚ ‚é‚¢‚Ķ‰»Šw—Ć–@ŒQ‚ÉŠ„‚č•t‚Æ‚é‚Ę‚¢‚¤‘O•ūŽ‹“I”äŠrŽŽŒ±‚ÅŒŸŲ‚³‚ź‚Ä‚«‚½B‚±‚Ģź‡CŽĄŪ‚ÉŠ„‚č•t‚Æ‚ē‚ź‚½Ž”—Ć‚Ŗs‚ķ‚ź‚Ä‚¢‚Č‚¢mĒ—į‚šŽĄŪ‚ɍs‚ķ‚ź‚½Ž”—Ć‚ÅŒQ•Ź‚µ‚ĉšĶ‚·‚é‚ĘƒoƒCƒAƒX‚š¶‚¶‚é‚Ģ‚ŁCŠ„‚č•t‚Æ‚ē‚ź‚½ŒQiƒhƒi[‚ ‚čŒQvs ƒhƒi[‚Č‚µŒQj‚ɏ]‚Į‚ĉšĶ‚³‚ź‚éiintent-to-treat analysisjnB“–‰‚ĢŒ¤‹†‚Ķ—\Œć•s—ĒˆöŽq‚š—L‚·‚éŒQ‚É‚Ø‚¢‚Ä‚Ģ‚Żƒhƒi[‚ ‚čŒQ‚Ģ¶‘¶ŠśŠŌ‚Ŗ‰„’·‚·‚é‚Ę‚¢‚¤•ń‚Ŗ‘½‚©‚Į‚½‚ŖC‚»‚ĢŒćC‹t‚ĢŒ‹‰Ź‚šŽ¦‚·‘å‹K–Ķ—Տ°Œ¤‹†‚ą•ń‚³‚ź‚Ä‚¢‚éB

‚±‚ź‚ē‚Ģ—Տ°ŽŽŒ±‚š“‡‚µ‚½ƒƒ^ƒAƒiƒŠƒVƒX‚ĢŒ‹‰Ź‚Å‚ĶC‘ęˆźŠ°‰šŠśALL ‘S‘Ģ‚Å‚Ģƒhƒi[‚ ‚čŒQ‚ĢŽ€–S‚Ģ‘Š‘ĪŠėŒÆ“x‚Ķ0.88i95“ CIF0.8-0.97Cp0.007j‚Ę—LˆÓ‚É’į‚­CƒTƒuƒOƒ‹[ƒv‰šĶ‚Å‚Ķ•W€ƒŠƒXƒNŒQ‚Å0.8i95“CIF0.68-0.94Cp0.006jC‚ƒŠƒXƒNŒQ‚Å0.88i95“CIF0.76-1.01Cp0.07j‚Ę•W€ƒŠƒXƒNŒQ‚Ģ‚Ż‚Å—LˆÓ·‚ŖŠĻŽ@‚³‚ź‚½1jB‚”N—ī‚Ŗ‚ƒŠƒXƒNŒQ‚Ģ’č‹`‚ÉŠÜ‚Ü‚ź‚Ä‚¢‚鎎Œ±‚Ŗ‘½‚¢‚ŖC”N—ī‚Ę‚¢‚¤ˆöŽq‚Ķ“ÆŽķˆŚA‚ʼnü‘P‚·‚é‚Ę‚¢‚¤‚±‚Ę‚Ķl‚¦‚É‚­‚­C”N—ī‚šˆŚA“K‰ž‚Ģ”»’fŽž‚ĢƒŠƒXƒN•Ŗ—Ž‚É—p‚¢‚é‚ׂ«‚Å‚Ķ‚Č‚¢‚Ę‚¢‚¤‚±‚Ę‚ŖŽw“E‚³‚ź‚Ä‚¢‚éB

JALSG ALL-93 ‚Ø‚ę‚ŃALL-97 ‚Ģ‰»Šw—Ć–@‚Ģƒf[ƒ^‚Ę“ś–{‘¢ŒŒ×–EˆŚAŠw‰ļ‚ĢˆŚAƒf[ƒ^‚š—p‚¢‚čs‚ķ‚ź‚½—Տ°Œˆ’f•ŖĶ‚Å‚ĶCHLA “K‡“Æ–E‚Ŗ‚¢‚鏼‡‚É‚Ķ‘ęˆźŠ°‰šŠś‚É“ÆŽķˆŚA‚šs‚¤Œˆ’f‚š‚·‚邱‚Ę‚Ģ—DˆŹ«‚ŖŽ¦‚³‚ź‚½2jBQOL •ā³‚šs‚Į‚½”äŠr‚Å‚ąC”N—ī‚É‚ę‚Į‚ÄŒQ•Ź‰»‚µ‚½‚·‚ׂĂĢƒTƒuƒOƒ‹[ƒv‚É‚Ø‚¢‚Ä‚ąˆŚAŒQ‚Ģ—DˆŹ«‚Ķ•Ļ‚ķ‚ē‚Č‚©‚Į‚½BHLA ACBCDRB1 “K‡‚Ģ”ńŒŒ‰ƒhƒi[‚©‚ē‚ĢˆŚAƒf[ƒ^‚š—p‚¢‚½‰šĶ‚Å‚ą‚Ł‚Ś“Æ—l‚ĢŒ‹‰Ź‚Ŗ“¾‚ē‚ź‚½B“K‡“x‚Ģ‚ę‚¢”ńŒŒ‰ŽŅŠŌˆŚA‚ĢŽ”—Ɛ¬Ń‚ĶHLA “K‡“Æ–E‚©‚ē‚ĢŽ”—Ɛ¬Ń‚Ę‚Ł‚Ś“Æ“™‚Å‚ ‚邱‚Ę‚Ŗ•ń‚³‚ź‚Ä‚¢‚é3jB‚µ‚©‚µC¬Ž™‰ČŒ^‚Ģ‰»Šw—Ć–@‚ĢĢ—p‚Č‚Ē‚É‚ę‚鉻Šw—Ć–@‚ĢŽ”—Ɛ¬Ń‚Ģ‰ü‘P‚É‚ę‚Į‚āC‚±‚ź‚ē‚ĢŒ‹˜_‚Ŗ‹t“]‚·‚é‰Ā”\«‚Ķ”Ū’č‚Å‚«‚Č‚¢B‚Ü‚½CHLA •s“K‡ˆŚA‚āä`‘ŃŒŒˆŚA‚Ģ“K‰ž‚š”»’f‚·‚邽‚ß‚Ģ–¾Šm‚Čƒf[ƒ^‚Ķ‚Č‚¢B«—ˆ“I‚É‚Ķ”÷¬Žc‘¶•a•Ļ‚šƒ‚ƒjƒ^[‚·‚邱‚Ę‚É‚ę‚Į‚āC“ÆŽķ‘¢ŒŒŠ²×–EˆŚA‚š•K—v‚Ę‚·‚銳ŽŅ‚š‚ę‚č³Šm‚É”»•Ź‚Å‚«‚é‚ꂤ‚É‚Č‚é‰Ā”\«‚ą‚ ‚éB

Ph —z«ALL ‚É‚Ā‚¢‚Ä‚Ķƒ`ƒƒVƒ“ƒLƒi[ƒ[‘jŠQÜ‚Ģ“±“ü‚É‚ę‚Į‚ĉ»Šw—Ć–@‚Ģ¬Ń‚Ŗ’˜‚µ‚­‰ü‘P‚µ‚Ä‚¢‚é‚ŖC‚»‚ĢŒų‰Ź‚Ŗ’·ŠśŠŌˆŪŽ‚³‚ź‚é‚©‚Ē‚¤‚©‚É‚Ā‚¢‚Ä‚Ķ•s–¾‚Å‚ ‚éBŒ»Žž“_‚Å‚Ķƒ`ƒƒVƒ“ƒLƒi[ƒ[‘jŠQÜ“±“üˆČ‘O‚Ģ—Տ°ŽŽŒ±‚Ģ¬Ń‚šŽQl‚É‚µ‚Ä4jC‘ęˆźŠ°‰šŠś‚Å‚Ģ“ÆŽķˆŚA‚ĢŽĄŽ{‚Ŗ„§‚³‚ź‚éB

yŽQl•¶Œ£z

1j Ram R, et al. Management of adult patients with acute lymphoblastic leukemia in first complete remission : systematic review and meta-analysis. Cancer. 2010 ; 116i14j : 3447-57.i1iiAj

2j Kako S, et al. A decision analysis of allogeneic hematopoietic stem cell transplantation in adult patients with Philadelphia chromosome-negative acute lymphoblastic leukemia in first remission who have an HLA-matched sibling donor. Leukemia. 2011 ; 25i2j : 259-65.i3iiAj

3j Kanda J, et al. Related transplantation with HLA 1-antigen mismatch in the graft-versus-host direction and HLA 8/8-allele-matched unrelated transplantation : A nationwide retrospective study. Blood. 2012 ; 119i10j : 2409-16.i3iiAj

4j Fielding AK, et al. Prospective outcome data on 267 unselected adult patients with Philadelphia chromosome-positive acute lymphoblastic leukemia confirms superiority of allogeneic transplantation over chemotherapy in the pre-imatinib era : results from the International ALL Trial MRC UKALLXII/ECOG2993. Blood. 2009 ; 113i19j : 4489-96.i1iiAj


CQ14

ALL ‚É‘Ī‚·‚éŒøŽć‘Oˆ’u‚É‚ę‚é“ÆŽķ‘¢ŒŒŠ²×–EˆŚA‚Ķ—L—p‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2B
’Źķ‚Ģ‹­“x‚ĢˆŚA‘Oˆ’u‚šs‚¤‚±‚Ę‚Ŗ‚Å‚«‚Č‚¢‚—īŽŅ‚ ‚é‚¢‚Ķ‘ŸŠķįŠQ‚š—L‚·‚é‘ęˆźŠ°‰šŠśALL Š³ŽŅ‚É‘Ī‚·‚éŒøŽć‘Oˆ’u‚É‚ę‚é“ÆŽķ‘¢ŒŒŠ²×–EˆŚA‚É‚ę‚Į‚āCˆź•”‚ĢŠ³ŽŅ‚É’·Šś¶‘¶‚Ŗ“¾‚ē‚ź‚Ä‚Ø‚čC‚»‚ĢŽĄŽ{‚šŒŸ“¢‚·‚é‚É’l‚·‚éB

y‰š ąz

‚—īŽŅALL ‚É‘Ī‚·‚鉻Šw—Ć–@‚Ģ¬Ń‚Ķ•s—Ē‚Å‚ ‚éBˆź•ūC‚—īŽŅ‚É‘Ī‚µ‚Ä’Źķ‚Ģ‹­“x‚Ģ‘Oˆ’uimyeloablative conditioningFMACj‚É‚ę‚é“ÆŽķ‘¢ŒŒŠ²×–EˆŚA‚ĢŽĄŽ{‚Ķ“ļ‚µ‚¢B‚»‚±‚ŁC‹­“x‚šŒøŽć‚µ‚½ˆŚA‘Oˆ’uireduced-intensity conditioningFRICj‚š—p‚¢‚éƒ~ƒjˆŚA‚ŖŽŽ‚Ż‚ē‚ź‚Ä‚¢‚éB“Į‚ÉUnited Kingdom Medical Research Council Adult Leukemia Working PartyiMRC UKALLjXII/ECOG 2992 ŽŽŒ±‚É‚Ø‚¢‚Ä35 ĪˆČć‚ĢŠ³ŽŅ‚ňŚAŠÖ˜AŽ€–S‚Ŗ‘‰Į‚µ‚½‚±‚Ę‚©‚ēCALL ‚É‘Ī‚·‚éƒ~ƒjˆŚA‚Ŗ’–Ś‚³‚ź‚é‚ꂤ‚É‚Č‚Į‚½B‚µ‚©‚µCŒ»Žž“_‚Å‚Ķƒ~ƒjˆŚA‚ʉ»Šw—Ć–@C‚ ‚é‚¢‚Ķƒ~ƒjˆŚA‚Ę’Źķ‚ĢˆŚA‚Ģ‘O•ūŽ‹“I”äŠrŽŽŒ±‚ĢŒ‹‰Ź‚Ķ“¾‚ē‚ź‚Ä‚¢‚Č‚¢B

MAC ‚ĘRIC ‚Ģ”äŠr‚É‚Ā‚¢‚Ä‚ĶEuropean Group for Blood and Marrow TransplantationiEBMTj‚ĘCenter for International Blood and Marrow Transplant ResearchiCIBMTRj‚©‚ē‚»‚ź‚¼‚ź‘å‹K–Ķ‚ČŒć•ūŽ‹“IŒ¤‹†‚Ŗ•ń‚³‚ź‚Ä‚¢‚éB‘OŽŅ‚Ķ45 ĪˆČć‚Ģ‘ęˆźC‘ę“ńŠ°‰šŠśŠ³ŽŅ‚š‘ĪŪ‚Ę‚µ‚Ä127 —į‚ĢRIC Ē—į‚Ę449 —į‚ĢMAC Ē—į‚š”äŠr‚µ‚½‚Ę‚±‚ėCRIC ŒQi”N—ī’†‰›’l56 ĪC”ĶˆĶ45`73 Īj‚ōĔ­‚Ŗ—LˆÓ‚É‘‰Į‚·‚é‚ą‚Ģ‚ĢˆŚAŠÖ˜AŽ€–SiTRMj‚Ķ—LˆÓ‚ÉŒø­‚µC–³”’ŒŒ•a¶‘¶Š„‡‚É‚Ķ·‚Ŗ‚Ż‚ē‚ź‚Č‚©‚Į‚½1jBŒćŽŅ‚ą‘ęˆźC‘ę“ńŠ°‰šŠśŠ³ŽŅ‚š‘ĪŪ‚Ę‚µ‚½93 —į‚ĢRIC Ē—įi”N—ī’†‰›’l45 ĪC”ĶˆĶ17`66 Īj‚Ę1,428 —į‚ĢMAC Ē—į‚Ģ”äŠr‚Å‚ ‚é‚ŖC‚±‚æ‚ē‚Ķ16 ĪˆČć‚ĢŠ³ŽŅ‚šŠÜ‚ń‚Å‚¢‚é2jB‘½•Ļ—Ź‰šĶ‚ĢŒ‹‰ŹC‘Oˆ’u‚Ģ‹­“x‚Ģˆį‚¢‚ĶÄ”­—¦C”ńÄ”­Ž€–S—¦C¶‘¶Š„‡‚É—LˆÓ·‚š—^‚¦‚Č‚©‚Į‚½BŒĀ•Ź‚Ģ‘Oˆ’u‚ÉŠÖ‚µ‚Ä‚Ķƒtƒ‹ƒ_ƒ‰ƒrƒ“iFLUj‚Ęƒƒ‹ƒtƒ@ƒ‰ƒ“iMELj‚Ģ‘g‚Ż‡‚ķ‚¹‚É‚ę‚é‘Oˆ’u‚Å—ĒD‚Ȑ¬Ń‚Ŗ“¾‚ē‚ź‚Ä‚¢‚é3j4jB

‰»Šw—Ć–@‚Ę‚Ģ—D—ņ‚Ķ•s–¾‚Å‚ ‚é‚ŖC‚—īŽŅALL ‚É‘Ī‚·‚é’Źķ‚Ģ‰»Šw—Ć–@‚ĢŽ”—Ɛ¬Ń‚Ķ•s—Ē‚Å‚ ‚邽‚߁C‚—īŽŅ‘ęˆźŠ°‰šŠśALL ‚É‘Ī‚·‚éƒ~ƒjˆŚA‚Ģ—L—p«‚š•]‰æ‚·‚é‘O•ūŽ‹“I”äŠrŽŽŒ±‚ĢŽĄŽ{‚ŖŠś‘Ņ‚³‚ź‚éB

yŽQl•¶Œ£z

1j Mohty M, et al. Reduced-intensity versus conventional myeloablative conditioning allogeneic stem cell transplantation for patients with acute lymphoblastic leukemia : a retrospective study from the European Group for Blood and Marrow Transplantation. Blood. 2010 ; 116i22j : 4439-43.i3iiAj

2j Marks DI, et al. The outcome of full-intensity and reduced-intensity conditioning matched sibling or unrelated donor transplantation in adults with Philadelphia chromosome-negative acute lymphoblastic leukemia in first and second complete remission. Blood. 2010 ; 116i3j : 366-74.i3iiAj

3j Stein AS, et al. Reduced-intensity conditioning followed by peripheral blood stem cell transplantation or adult patients with high-risk acute lymphoblastic leukemia. Biol Blood Marrow Transplant. 2009 ; 15i11j : 1407-14.i3iiAj

4j Cho BS, et al. Reduced-intensity conditioning allogeneic stem cell transplantation is a potential therapeutic approach for adults with high-risk acute lymphoblastic leukemia in remission : results of a prospective phase 2 study. Leukemia. 2009 ; 23i10j : 1763-70.i3iiAj


CQ15

ALL Ä”­—įiPh ‰A«‘O‹ģB ×–EALLCPh —z«‘O‹ģB ×–EALLC‘O‹ģT ×–EALLj‚É‘Ī‚·‚éÄŠ°‰š“±“ü—Ć–@‚Ģ‘I‘šŽˆ‚Ę‚µ‚ĉ½‚ŖŠ©‚ß‚ē‚ź‚é‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2B
ALL Ä”­—į‚Å‚Ķ‘OŽ”—Ć—š‚šl—¶‚µ‚½ÄŠ°‰š“±“ü—Ć–@‚šs‚¤B”ÓŠśÄ”­—į‚Å‚Ķ‰‰ńŠ°‰š“±“ü—Ć–@‚Ę“Æˆź‚ĢƒŒƒWƒƒ“‚É‚ę‚éÄŽ”—Ć‚ą‘I‘šŽˆ‚É“ü‚éB

„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2A
Ph —z«ALL ‚ĢƒCƒ}ƒ`ƒjƒuŒp‘±’†‚ĢÄ”­‚Å‚Ķƒ_ƒTƒ`ƒjƒu‚Ö‚Ģ•ĻX‚Ŗ‘Ć“–‚Å‚ ‚éB

„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2B
‘O‹ģT ×–EALL ‚Å‚Ķƒlƒ‰ƒ‰ƒrƒ“‚ŖŽ”—Ć‘I‘šŽˆ‚ɉĮ‚ķ‚éB

y‰š ąz

¬lALL ‚ĢÄ”­—į‚Ģ—\Œć‚Ķˆź”Ź‚É•s—Ē‚Å‚ ‚é‚ŖCÄŠ°‰š“±“ü—Ć–@Œć‚É“ÆŽķ‘¢ŒŒŠ²×–EˆŚA‚Ŗ‰Ā”\‚ČŠ³ŽŅ‚Å‚Ķ‚±‚ź‚É‚ę‚é—\Œć‰ü‘P‚ŖŠś‘Ņ‚Å‚«‚éBALL ‚ĢÄ”­‚Ķ’nŒÅ‚ß—Ć–@’†CˆŪŽ—Ć–@’†CˆŪŽ—Ć–@I—¹Œć‚Č‚Ē‚³‚Ü‚“‚Ü‚ČŽžŠś‚É‹N‚±‚č“¾‚é‚ŖCÄ”­ŽžŠś‚ā‘OŽ”—Ć—š‚É‚ę‚Į‚čĊ°‰š“±“ü—Ć–@‚Ģ“ą—e‚Ŗl—¶‚³‚ź‚éBAdVP —Ć–@iDXR, VCR, PSLj‚āhyper-CVAD —Ć–@iCPA, VCR, DXR, DEXj‚É‘ć•\‚³‚ź‚éƒAƒ“ƒgƒ‰ƒTƒCƒNƒŠƒ“ŒnRŽīį‡–ņCƒrƒ“ƒNƒŠƒXƒ`ƒ“iVCRjCƒXƒeƒƒCƒh–ņ•¹—p—Ć–@1jCL-ƒAƒXƒpƒ‰ƒMƒi[ƒ[iL-Aspj‚šŠÜ‚Ž‘½Ü•¹—p—Ć–@2jC‘å—ŹƒVƒ^ƒ‰ƒrƒ“—Ć–@‚šŠÜ‚Ž‘½Ü•¹—p—Ć–@3j‚Č‚Ē‚ŖÄ”­ALL ‚É‘Ī‚·‚éÄŠ°‰š“±“ü—Ć–@‚Ę‚µ‚ÄŽ”—Ɛ¬Ń‚Ŗ•ń‚³‚ź‚Ä‚¢‚éBÄ”­ŽžŠś•Ź‚É•Ŗ‚ƂčĊ°‰š“±“ü—Ć–@‚š‘½”—į‚ÅŒŸ“¢‚µ‚½—Տ°ŽŽŒ±‚Ķ•ń‚³‚ź‚Ä‚¢‚Č‚¢BÄ”­ALL ‚É‘Ī‚·‚éÄŠ°‰š“±“ü—Ć–@‚ĢŠ®‘SŠ°‰šiCRj—¦‚Ķ‘S‘Ģ‚Ę‚µ‚Ä50“‚š‰ŗ‰ń‚čC“Į‚ÉŠ°‰šŠśŠŌ‚Ŗ1 ”N–¢–ž‚ĢŠ³ŽŅ‚Å‚Ķ‚³‚ē‚É’į‚¢‚Ę‚³‚ź‚é4jB

ƒCƒ}ƒ`ƒjƒuŒp‘±’†‚ĢPh —z«ALL ‚ĢŠ³ŽŅ‚ĢÄ”­—į‚š‘ĪŪ‚Ę‚µ‚½ƒ_ƒTƒ`ƒjƒu’PÜ—Ć–@‚Ģ‘ęⅡ‘ŠŽŽŒ±‚Å‚ĶCŒŒ‰tŠw“I‘tŒų—¦‚Ŗ42“‚ŁC”ƒJŒŽŠŌ‘tŒų‚ŖˆŪŽ‚Å‚«‚邱‚Ę‚ŖŽ¦‚³‚ź‚Ä‚¢‚é5jBƒCƒ}ƒ`ƒjƒu’ļR«•ĻˆŁ‚šŠl“¾‚µ‚½Š³ŽŅ‚Ģˆź•”‚Å‚Ķƒ_ƒTƒ`ƒjƒu‚Ŗ—LŒų‚Å‚ ‚邱‚Ę‚ŖŽ¦“‚³‚ź‚éB‚Č‚ØPh —z«ALL ‚ĢÄ”­—į‚É‚Ø‚Æ‚éƒ_ƒTƒ`ƒjƒu•¹—p‰»Šw—Ć–@‚Ģ—LŒų«‚ĘˆĄ‘S«‚Ķ–¾‚ē‚©‚Å‚Ķ‚Č‚¢B

T ×–E«ALLiT-ALLj‚ĢÄ”­E“ļŽ”—į‚Å‚Ķƒlƒ‰ƒ‰ƒrƒ“iAraGj—Ć–@‚Ģ—LŒų«‚ŖŽ¦‚³‚ź‚Ä‚¢‚éBCancer and Leukemia Study Group BiCALGBj‚Ŗs‚Į‚½‘ęⅡ‘ŠŽŽŒ±‚ŁC‘½‚­‚Ģ‘OŽ”—Ć—š‚š—L‚·‚éT-ALL ‚ĢŠ³ŽŅ‚É‚Ø‚¢‚Ä‘S‘tŒų—¦‚Ŗ41“‚Å‚ ‚Į‚½6jB

yŽQl•¶Œ£z

1j Koller CA, et al. The hyper-CVAD regimen improves outcome in relapsed acute lymphoblastic leukemia. Leukemia. 1997 ; 11i12j : 2039-44.i3iiiDivj

2j Esterhay RJ, Jr., et al. Moderate dose methotrexate, vincristine, asparaginase, and dexamethasone for treatment of adult acute lymphocytic leukemia. Blood. 1982 ; 59i2j : 334-45.i3iiDivj

3j Kantarjian HM, et al. Mitoxantrone and high-dose cytosine arabinoside for the treatment of refractory acute lymphocytic leukemia. Cancer. 1990 ; 65i1j : 5-8.i3iiiDivj

4j Tavernier E, et al. Outcome of treatment after first relapse in adults with acute lymphoblastic leukemia initially treated by the LALA-94 trial. Leukemia. 2007 ; 21i9j : 1907-14.i3iDiij

5j Ottmann O, et al. Dasatinib induces rapid hematologic and cytogenetic responses in adult patients with Philadelphia chromosome positive acute lymphoblastic leukemia with resistance or intolerance to imatinib : interim results of a phase 2 study. Blood. 2007 ; 110i7j : 2309-15.i3iDivj

6j DeAngelo DJ, et al. Nelarabine induces complete remissions in adults with relapsed or refractory T-lineage acute lymphoblastic leukemia or lymphoblastic lymphoma : Cancer and Leukemia Group B study 19801. Blood. 2007 ; 109i12j : 5136-42.i3iDivj


4
  –«œ‘«”’ŒŒ•a/ œ‘‘B«Žīį‡
ichronic myelogenous leukemia : CML
    /myeloproliferative neoplasms : MPNj

Ÿ‘˜_

œ‘‘B«Žīį‡imyeloproliferative neoplasmsFMPNj‚ĶC‘¢ŒŒŠ²×–EƒŒƒxƒ‹‚Å‚ĢŽīį‡‰»‚É‚ę‚Į‚Ä”­Ē‚·‚鎾Š³‚Å‚ ‚čCœ‘Œn×–Eič÷—±‹…CŌ‰č‹…Cœ‘‹Šj‹…C”ģ–ž×–Ej‚Ģ’˜‚µ‚¢‘B‚š“Į’„‚Ę‚·‚é1jBMPN ‚É‚ĶC–«œ‘«”’ŒŒ•aichronic myelogenous leukemiaFCMLjC–«D’†‹…«”’ŒŒ•aichronic neutrophilic leukemiaFCNLjC^«ŌŒŒ‹…‘‰ĮĒ‚Ü‚½‚Ķ^«‘½ŒŒĒipolycythemia veraFPVjCŒ“”­«œ‘üˆŪĒiprimary myelofibrosisFPMFjC–{‘Ō«ŒŒ¬”ĀŒŒĒiessential thrombocythemiaFETjC–«DŽ_‹…«”’ŒŒ•aichronic eosinophilic leukemiaFCELjCDŽ_‹…‘‰ĮĒŒóŒQihypereosinophilic syndromeFHESjC”ģ–ž×–EĒimastocytosisjC•Ŗ—Ž•s”\œ‘‘B«Žīį‡imyeloproliferative neoplasmsCunclassifiableFMPN, Uj‚ŖŠÜ‚Ü‚ź‚éB”­Ē‰Šś‚ĢMPN ‚ĶC•Ŗ‰»”\‚š—L‚·‚鍜‘×–E‚Ģ‰ßŒ`¬‚ʁC––½ŒŒ‚É‚Ø‚Æ‚éč÷—±‹…CŌŒŒ‹…CŒŒ¬”Ā‚Ģ‘‰Į‚šŽ¦‚·B—Šw“I‚É‚ĶäBŽī‚āŠĢŽķ‘å‚š”F‚ß‚éBMPN ‚Ķ”­ĒŽžŠśCŽ©ŠoĒó‚É–R‚µ‚¢‚ŖC‘SgĒó‚š”ŗ‚¢’iŠK“I‚É‘ˆ«‚µCÅI“I‚É‚Ķœ‘‚ĢüˆŪ‰»C‚ ‚é‚¢‚ĶCŒ`Žæ“]Š·‚µ‚Ьn”\‘rŽøi‹}«“]‰»j‚ÖŽŠ‚čCœ‘•s‘S‚Ę‚¢‚¤’vŽ€“I‚ȏI––Šś‚ւʐis‚·‚éBMPN ‚ĢŽ”—Ć‚É‚Ā‚¢‚Ä‚ĶCCML ‚Ę‚»‚źˆČŠO‚ĢMPN ‚Å‚Ķ•ūj‚ŖˆŁ‚Č‚éB–{ƒKƒCƒhƒ‰ƒCƒ“‚Å‚ĶCMPN ‚Ģ‚¤‚æŽå‚ÉCML ‚ĘPVCETC‚»‚µ‚ÄPMF ‚ĢŽ”—Ć‚š’ńŽ¦‚·‚éB

1D–«œ‘«”’ŒŒ•aichronic myelogenous leukemiaFCMLj

1j•aŠś

CML ‚ĶC‘½”\«‘¢ŒŒŠ²×–E‚ĢˆŁķ‚É‚ę‚čŽä‹N‚³‚ź‚é”’ŒŒ•a‚Åt (9;22)(q34;q11) ‚É‚ę‚čŒ`¬‚³‚ź‚éPhiladelphiaiPhjõF‘Ģ‚š“Į’„‚Ę‚·‚éBPh õF‘Ģć‚ĢBCR-ABL1 —Z‡ˆā“`Žq‚ɃR[ƒh‚³‚ź‚ÄŽY¶‚³‚ź‚éBCR-ABL ƒ`ƒƒVƒ“ƒLƒi[ƒ[ityrosine kinaseFTKj‚ŖPķ“I‚ÉŠˆ«‰»‚µC”’ŒŒ•a×–E‚Ģ‘B‚ÉŠÖ—^‚µC3 ‚Ā‚Ģ•aŠś‚šŒo‚Đis‚·‚é2jBCML ‚ĶC”’ŒŒ‹…‚āŒŒ¬”Ā‚Ģ‘‰Į‚š”F‚ß‚é‚ŖŽ©ŠoĒó‚Ģ–R‚µ‚¢–«Šśichronic phaseFCPCf’fŒć–ń3`5 ”NŠŌj‚Å‘½‚­‚ĢŠ³ŽŅi85“j‚Ŗf’f‚³‚źCč÷—±‹…‚Ģ•Ŗ‰»ˆŁķ‚Ŗis‚·‚éˆŚsŠśiaccelerated crisisFAPC3`9 ƒJŒŽŠŌj‚šŒo‚āC–¢•Ŗ‰»‚ȉ苅‚Ŗ‘‰Į‚µ‚Ä‹}«”’ŒŒ•a‚É—ŽŽ—‚·‚é‹}«“]‰»Šśiblast phaseFBPC–ń3`6 ƒJŒŽj‚֐i“W‚µ’vŽ€“I‚Ę‚Č‚éBWHO •Ŗ—Ži2008j‚Ģ‹K€‚ɏ]‚¢CPCAPCBP Šś‚Ŗ’č‹`‚³‚ź‚éi•\1jB

2jCML ‚Ģ—\Œć•Ŗ—Ž

‰fŽž‚Ģ”N—īCäBŽīi˜]œ‹|‰ŗcmjCŒŒ¬”Ā”C––½ŒŒ‰č‹…i“j‚Ģ4 ˆöŽq‚š—p‚¢‚ÄŒvŽZ‚³‚ź‚éSokal ƒXƒRƒA3j‚āC”N—īCäBŽīi˜]œ‹|‰ŗcmjC––½ŒŒ‰č‹…i“jC––½ŒŒDŽ_‹…”i“jC––½ŒŒD‰–Šī‹…i“jCŒŒ¬”Ā”‚Ģ6 ˆöŽq‚š—p‚¢‚ÄŒvŽZ‚³‚ź‚éHasford ƒXƒRƒA4j‚ĶC‚±‚ź‚Ü‚Å‚ą‰»Šw—Ć–@‚āƒCƒ“ƒ^[ƒtƒFƒƒ“ƒæiIFNƒæj—Ć–@Žž‘ć‚É—p‚¢‚ē‚ź‚Ä‚«‚½‚ŖCƒCƒ}ƒ`ƒjƒuŽ”—Ć‚É‚Ø‚¢‚Ä‚ą—L—p‚Å‚ ‚čCLowCIntermediateCHigh ‚Ģ3 ƒŠƒXƒNŒQ‚É•Ŗ—Ž‚³‚ź‚éijBƒCƒ}ƒ`ƒjƒuŽ”—ĆŠ³ŽŅ‚š‘ĪŪ‚Ę‚µ‚½‰šĶ‚ę‚č\’z‚³‚ź‚½—\Œć—\‘ŖƒVƒXƒeƒ€EUTOS ƒXƒRƒA5j ‚ĶC ‰fŽž‚ĢD‰–Šī‹…i“j ‚ĘäBŽī‚Ģ‚Ż‚ÅŒvŽZ‚³‚źi7~basophils“{4~spleen size cmjC87 ˆČ‰ŗ‚ĢLow ‚Ę87 ‚ę‚č‘å‚«‚¢High ‚Ģ2 ƒŠƒXƒNŒQ‚Ŗ’ń„‚³‚ź‚Ä‚¢‚éijB

•\1@WHO •Ŗ—Ži2008j‚É‚ę‚éCML ‚Ģ•aŠś•Ŗ—Ž

–«Šśichronic phasej

ˆČ‰ŗ‚ĢˆŚsŠśC‹}«‰č‹…“]‰»Šś‚š–ž‚½‚³‚Č‚¢‚ą‚Ģ

ˆŚsŠśiaccelerated phasej

ˆČ‰ŗ‚Ģ‚¢‚ø‚ź‚©‚Š‚Ę‚Ā‚ÉŠY“–‚·‚é‚ą‚Ģ

––½ŒŒ‚ ‚é‚¢‚Ķœ‘‚É‚Ø‚Æ‚é‰č‹…Š„‡
10`19“
––½ŒŒ‚É‚Ø‚Æ‚éD‰–Šī‹…Š„‡
†20“
ŒŒ¬”Ā”
Ž”—Ć‚É–³ŠÖŒW‚ĢŒŒ¬”ĀŒø­iƒ10,000/ƒŹLj
Ž”—Ć‚Ŗ‘tŒų‚µ‚Č‚¢ŒŒ¬”Ā‘‰Įi„1,000,000/ƒŹLj
”’ŒŒ‹…”‚Ø‚ę‚ŃäBŽī
Ž”—Ć‚Ŗ‘tŒų‚µ‚Č‚¢Ž‘±‚·‚é”’ŒŒ‹…‘‰Įi„10,000/ƒŹLj
}Ž‘±‚ ‚é‚¢‚Ķ‘‹­‚·‚éäBŽī
õF‘ĢˆŁķ
•t‰Į“I‚ȐõF‘ĢˆŁķ‚Ģ”­Œ»

‹}«“]‰»ŠśiBlast phasej

‰ŗ‹L‚Ģ‚¢‚ø‚ź‚©‚Š‚Ę‚Ā‚ÉŠY“–‚·‚é‚ą‚Ģ

––½ŒŒ‚ ‚é‚¢‚Ķœ”Õ‚É‚Ø‚Æ‚é‰č‹…Š„‡
†20“
‘ŠOZ
‘ŠO•a•Ļ‚ĢoŒ»
œ‘¶ŒŸ•W–{‚Å‰č‹…‚Ģ‘å‚«‚ȏWĻ‘œ‚š”F‚ß‚é
3jCML ‚ĢŽ”—ĆŒų‰Ź”»’č

CML Ž”—Ć‚ĢƒRƒ“ƒZƒvƒg‚ĶPh —z«iBCR-ABL1 —z«j”’ŒŒ•a×–E‚ĢƒRƒ“ƒgƒ[ƒ‹‚Ę•aŠśis‚Ģ‰ń”š‚É‚ ‚čCŽ”—ĆŒų‰Ź‚ĶEuropean LeukemiaNet (ELN) ‚Ģ”»’č‹K€‚ɏ]‚¤6j7ji•\2jB

CP Šś‚ĢŽ”—ĆŒų‰Ź‚ĶCŒŒ‰tŠw“I‘tŒųihematologic responseFHRjC×–Eˆā“`Šw“I‘tŒųicytogenetic responseFCyRjC•ŖŽqˆā“`Šw“I‘tŒųimolecular responseFMRj‚Ģ3 ‚Ā‚ĢƒŒƒxƒ‹‚Å”»’č‚·‚éi•\2jBHR ‚Ķ––½ŒŒŠŒ©‚Ģ‰ü‘PCCyR ‚Ķœ‘×–E’†‚ĢPh õF‘ĢŠ„‡‚ŁCMR ‚Ķƒ|ƒŠƒƒ‰[ƒ[˜A½”½‰žipolymerase chain reactionFPCRj‚É‚ę‚čŒŒ‰t×–E’†‚ĢBCR-ABL1 ˆā“`Žq”­Œ»—Ź‚Å”»’f‚³‚ź‚éBAP/BP Šś‚Å‚ĶCŒŒ‰tŠw“I‘tŒų‹K€‚ŖCP Šś‚ĘˆŁ‚Č‚é‚ŖCCyR ‚ĘMR ‚Ķ“Æ‚¶‹K€‚š—p‚¢‚éB

•\2@–«œ‘”’ŒŒ•a‚É‘Ī‚·‚鎔—ĆŒų‰Ź‚Ģ”»’č‹K€
ŒŒ‰tŠw“I‘tŒųiHematologic ResponseFHRj ŒŒ‰tEœ‘ŒŸøŠŒ©‚Ø‚ę‚Ń—Õ°ŠŒ©
–«ŠśCML Š®‘SicompletejHRFCHR
  1. WBCƒ10,000/ƒŹL
  2. PLTƒ450,000/ƒŹL
  3. ––½ŒŒ‰t’†‚Å‰č‹…‚ą‘Oœ‘‹…‚ą‚Č‚µ
  4. ––½ŒŒ‰t’†‚Ģœ‘‹…{Œćœ‘‹…ƒCML Ž”—ĆŒų‰Ź‚Ģƒ‚ƒjƒ^ƒŠƒ“ƒO5“
  5. D‰–Šī‹…ƒ5“
  6. äB‘Ÿ‚Ø‚ę‚ŃŠĢ‘Ÿ‚ĢŽī‘å‚Č‚­C‘ŠO•a•Ļ‚Č‚µ
isŠśCML
iˆŚsŠś{‹}«Šśj
Š®‘SicompletejHRFCHR
  1. WBC…Ž{ŻŠī€’l‚ĢćŒĄ
  2. D’†‹…”†1,000/ƒŹL
  3. PLT†100,000/ƒŹL
  4. ––½ŒŒ‰t’†‚Å‰č‹…‚ą‘Oœ‘‹…‚ą‚Č‚µ
  5. œ‘’†‚Ģ‰č‹……5“
  6. ––½ŒŒ‰t’†‚Ģœ‘‹…{Œćœ‘‹…ƒ5“
  7. D‰–Šī‹…ƒ20“
  8. äB‘Ÿ‚Ø‚ę‚ŃŠĢ‘Ÿ‚ĢŽī‘å‚Č‚­C‘ŠO•a•Ļ‚Č‚µ
”’ŒŒ•a‚ĢŠŒ©‚Č‚µF
No Evidence of LeukemiaiNELj
  1. WBC…Ž{ŻŠī€’l‚ĢćŒĄ
  2. ––½ŒŒ‰t’†‚Å‰č‹…‚ą‘Oœ‘‹…‚ą‚Č‚µ
  3. œ‘’†‚Ģ‰č‹……5“
  4. ––½ŒŒ‰t’†‚Ģœ‘‹…{Œćœ‘‹…ƒ5“
  5. D‰–Šī‹…ƒ20“
  6. äB‘Ÿ‚Ø‚ę‚ŃŠĢ‘Ÿ‚ĢŽī‘å‚Č‚­C‘ŠO•a•Ļ‚Č‚µ
×–Eˆā“`Šw“I‘tŒųiCytogenetic ResponseFCyRj œ‘—LŠj×–E’†‚ĢPh õF‘ĢiBCR-ABLj—z«—¦

×–Eˆā“`Šw“I‘åimajorj‘tŒųFMCyR
×–Eˆā“`Šw“IŠ®‘Sicompletej‘tŒųFCCyR
×–Eˆā“`Šw“I•”•Ŗipartialj‘tŒųFPCyR

0 `35“
0“
1 `35“
×–Eˆā“`Šw“I¬iminorj‘tŒųFMinor CyR
36 `65“
×–Eˆā“`Šw“I”÷¬iminimumj‘tŒųFMini CyR
66 `95“
×–Eˆā“`Šw“I”ńinonej‘tŒųFNo CyR
„95“
•ŖŽqˆā“`Šw“I‘tŒųiMolecular ResponseFMRj BCR-ABL1 ˆā“`ŽqƒŒƒxƒ‹iRT-PCR –@j
•ŖŽqˆā“`Šw“I‘åimajorj‘tŒųFMMR BCR-ABL1IS–2 … 0.1“

•ŖŽqˆā“`Šw“I‚É”’ŒŒ•a–¢ŒŸo
MR4.0–1

MR4.5

BCR-ABL1IS–2 … 0.01“C
‚Ü‚½‚ĶABL1 ˆā“`ŽqcDNA „ 10,000 ƒRƒs[’†–¢ŒŸo
BCR-ABL1IS–2 … 0.0032“C
‚Ü‚½‚ĶABL1 ˆā“`ŽqcDNA „ 32,000 ƒRƒs[’†–¢ŒŸo

–1 ELN2009 ‚Å‚Ķ•ŖŽqˆā“`Šw“IŠ®‘Sicompletej‘tŒųiCMRj‚Ę’č‹`‚³‚ź‚½‘tŒųƒŒƒxƒ‹
–2 BCR-ABL1ISF‘ŪŽw•W‚Å•ā³‚³‚ź‚½’l

4jCML ‚ĢŽ”—ĆŠT—Ŗ
  1. BCR-ABL1 ƒ`ƒƒVƒ“ƒLƒi[ƒ[‘jŠQÜ—Ć–@FBCR-ABL1 ƒ`ƒƒVƒ“ƒLƒi[ƒ[‚š‘I‘š“I‚É‘jŠQ‚µCŒŒ‰tŠw“IC×–Eˆā“`Šw“IC•ŖŽqˆā“`Šw“I‚É—D‚ź‚½—LŒų«‚šŽ¦‚·ƒ`ƒƒVƒ“ƒLƒi[ƒ[‘jŠQÜityrosine kinase inhibitorFTKIj‚É‚ĶCƒCƒ}ƒ`ƒjƒu8j`11j‚Ģ‚Ł‚©Cƒjƒƒ`ƒjƒu12j‚Ęƒ_ƒTƒ`ƒjƒu13j‚Ŗ—Տ°“I‚É—p‚¢‚ē‚ź‚éBƒCƒ}ƒ`ƒjƒu‚ĶIFNƒæ{’į—p—ŹƒVƒ^ƒ‰ƒrƒ“iAraCj‚Ę‚Ģ”äŠrŽŽŒ±‚Ģ5 ”N-’·Šś¬Ń‚ĢŒ‹‰ŹCIFNƒæ‚É‘Ö‚ķ‚Į‚­CML-CP Šś‚É‘Ī‚·‚é‘ęˆź‘I‘š–ņ‚Ę‚Č‚Į‚½9jB“ś–{l‚É‚Ø‚¢‚Ä‚ąCƒCƒ}ƒ`ƒjƒuŽ”—Ć‚Ģ—D‚ź‚½’·Šś¬Ń‚ŖŠm”F‚³‚ź‚½11jB
  2. ƒjƒƒ`ƒjƒu‚Ęƒ_ƒTƒ`ƒjƒu‚ĶCƒCƒ}ƒ`ƒjƒuŽ”—Ć‚É’ļR«E•s‘Ļ—e‚ĢCML ‚É‘Ī‚·‚鎔—Ć–ņ‚Ę‚µ‚ÄŠJ”­‚³‚ź‚½‘ę“ń¢‘ćTKI ‚Å‚ ‚é‚ŖCƒCƒ}ƒ`ƒjƒu‚Ę‚Ģ”äŠrŽŽŒ±‚ĢŒ‹‰Ź12j13jC‰”­CML-CP Šś‚ĢŽ”—Ć‚Ę‚µ‚Ä‚ą‘I‘š‚Å‚«‚éBi‘ę1.1 ”Å’Ē‰ĮƒRƒƒ“ƒgj‚Ü‚½AV‹K‘ę“ń¢‘ćTKI ‚Ģƒ{ƒXƒ`ƒjƒu‚ĶAę‚É“Š—^‚µ‚½TKIiƒCƒ}ƒ`ƒjƒuAƒjƒƒ`ƒjƒuA‚Ü‚½‚Ķƒ_ƒTƒ`ƒjƒujŽ”—Ć‚É’ļR«E•s‘Ļ—e‚ĢCML ‚É‘Ī‚·‚鎔—Ć–ņ‚Å‚ ‚é32jB
  3. “ÆŽķ‘¢ŒŒŠ²×–EˆŚAiallogeneic stem cell transplantationFallo-HSCTjFŖŽ”‚ŖŠś‘Ņ‚Å‚«‚鎔—Ć–@‚Å‚ ‚é‚ŖCŽ”—ĆŠÖ˜A“Ő«‚Ģ‚½‚ß‚É‘ŠśŽ€–S‚ĢƒŠƒXƒN‚Ŗ‚‚¢BCML-CP ‚©‚ēis‚µ‚½TKI ‘Ļ«‚ĢAP/BP Šś‚ā‰”­ŽžAP/BP Šś‚É“K‰ž‚Ŗ‚ ‚éB‚Ü‚½C“KŲ‚Čƒhƒi[‚ĢŠm•ŪCˆŚAŠÖ˜A“Ő«‚É‘Ļ‚¦“¾‚é”N—ī“I‚Ø‚ę‚Ń‘Sgó‘Ō‚Å‚ ‚邱‚Ę‚Č‚Ē“K‰ž‚šl—¶‚·‚é14jB
  4. IFNƒæFIFNƒæ’PÜ15j‚ ‚é‚¢‚Ķ’į—p—ŹAraC ‚Ę‚Ģ•¹—p9j‚ĶƒCƒ}ƒ`ƒjƒuˆČ‘O‚Ģ•W€—Ć–@‚Å‚ ‚čCˆź•”‚ĢĒ—į‚ÅPh õF‘Ģ‚ĢĮŽø‚š”F‚߁C‘S¶‘¶ŠśŠŌioverall survivalFOSj‚Ģ‰ü‘P‚ÉŠń—^‚·‚邱‚Ę‚Ŗ’m‚ē‚ź‚Ä‚¢‚éB
  5. ‚»‚Ģ‘¼‚Ģ–ņ•Ø—Ć–@Fć‹LŽ”—ĆˆČŠO‚É‘I‘š‚³‚ź‚鎔—Ć–@‚Å‚ ‚é‚ŖCPh õF‘Ģ—z«×–E‚Ģ\•Ŗ‚ȏœ‹Ž‚Ķ¢“ļ‚Å‚ ‚čCĒóŠÉ˜a“I‚ČŽ”—Ć–@‚Å‚ ‚éBƒqƒhƒƒLƒVƒEƒŒƒAiHUjCƒuƒXƒ‹ƒtƒ@ƒ“iBUjC’į—p—ŹAraC ‚ŖŠÜ‚Ü‚ź‚éB‰‰ńŽ”—Ć‚ÉBU ‚Ķ„§‚³‚ź‚Č‚¢‚ŖCHU ‚ĶCML ‚Ģf’f‚Ŗ‚Ā‚­‚Ü‚Å’ZŠśŠŌ“Š—^‚³‚ź‚邱‚Ę‚Ŗ‚ ‚éB
5jCML Ž”—ĆŒų‰Ź‚Ģƒ‚ƒjƒ^ƒŠƒ“ƒO

European LeukemiaNet 2009 6j‚Ø‚ę‚щü’ł‚³‚ź‚½2013 7j‚ɏ]‚¢CƒCƒ}ƒ`ƒjƒu‚Č‚Ē‚ĢTKI —Ć–@‚Ģƒ‚ƒjƒ^ƒŠƒ“ƒO‚šs‚¤6j7jBŽ”—ĆŒų‰Ź‚Ģ”»’č•ū–@‚ĶCCyR ‚ĶCœ‘×–E‚ĢõF‘ĢŒŸøˆČŠO‚É––½ŒŒ‰t×–E‚ĢŒuŒõ in situ ƒnƒCƒuƒŠƒ_ƒCƒ[[ƒVƒ‡ƒ“ifluorescence in situ hybridizationFFISHj‚Å”»’č‚Å‚«‚éBMR ‚ĶC––½ŒŒ‰t‚š—p‚¢‚Ä’č—Źiquantitativej‹t“]ŽŹƒ|ƒŠƒƒ‰[ƒ[˜A½”½‰žiRT-PCRj‚ÅBCR-ABL1 ˆā“`ŽqƒŒƒxƒ‹‚šABL ‚ ‚é‚¢‚Ķ‘ĪŪ‚Ę‚Č‚éˆā“`Žq‚Ģ”ä‚šC‘ŪŽw•WiInternational ScaleFISj‚Å•ā³‚µ‚ÄBCR-ABL1IS ‚Ę•\‚·B‰‰ńi1st linejŽ”—Ć‚Å‚ĶCŽ”—ĆŒć3 ƒJŒŽ‚Ü‚Å‚ÉBCR-ABL1IS…10“‚Ü‚½‚Ķ•”•ŖCyRipartial CyRFPCyRjC6 ƒJŒŽ‚Ü‚Å‚ÉBCR-ABL1IS ƒ 1“‚Ü‚½‚ĶŠ®‘SCCyRicomplete CyRFCCyRjC12 ƒJŒŽ‚Ü‚Å‚ÉBCR-ABL1IS…0.1“‚·‚Č‚ķ‚æMajor MRiMMRjC‚»‚źˆČŒć‚ĶBCR-ABL1IS…0.1“‚šˆŪŽ‚·‚éOptimaliŽŠ“Kj‚ČŒų‰Ź‚š“¾‚邱‚Ę‚š–ŚŽw‚·i•\3-1jB‚»‚µ‚āCWarningi—v’ˆÓj‚Å‚Ķƒ‚ƒjƒ^ƒŠƒ“ƒO‚š•p‰ń‚ɍs‚¢CFailureiŽ”—Ć‚ĢŽø”sj‚Å‚ĶCŽ”—Ć‚Ģ•ĻX‚šl—¶‚·‚éB

‰‰ńŽ”—Ć‚ĢƒCƒ}ƒ`ƒjƒu‚©‚ē‘ę2 ¢‘ćTKI ‚É•ĻX‚µ‚½ź‡‚ĶC3 ƒJŒŽ‚ÅBCR-ABL1IS…10“‚Ü‚½‚ĶPh —z«×–Eƒ 65“C6 ƒJŒŽ‚ÅBCR-ABL1IS…10“‚Ü‚½‚ĶMCyRC12 ƒJŒŽ‚ÅBCR-ABL1IS…1“‚Ü‚½‚ĶCCyRC‚»‚źˆČ~BCR-ABL1IS…0.1“‚šŽŠ“K‘tŒų‚Ę‚µ‚Ä‚¢‚éi•\3-2jB>/p>

ELN 2009 ƒRƒ“ƒZƒ“ƒTƒX6j‚Å‚ĶC•ŖŽqˆā“`Šw“IŠ®‘S‘tŒųiCMRj‚šBCR-ABL1 ŒŸoŠ““xˆČ‰ŗ‚Ę’č‹`‚³‚ź‚½‚ŖCELN 2013 ƒRƒ“ƒZƒ“ƒTƒX7j‚Å‚ĶCBCR-ABL1IS 0.01“ˆČ‰ŗ‚šMR4CBCR-ABL1IS 0.0032“ˆČ‰ŗ‚šMR4.5CBCR-ABL1IS 0.001“ˆČ‰ŗ‚šMR5 ‚Ę‚µ‚½‹K€16j‚šĢ—p‚µ‚Ä‚¢‚éBCML-CP Ž”—Ć‚É‚Ø‚¢‚Ä‚ĶC­‚Č‚­‚Ę‚ąMMR ‚ĢŽ”—ĆŒų‰Ź‚š“¾‚邱‚Ę‚Ŗ‘åŲ‚Å‚ ‚čC’č—ŹRT-PCR ‚ĢŒŸõ‚ĶELN ‚āNCCN ‚Č‚ĒŠCŠO‚ĢCML Ž”—ĆƒKƒCƒhƒ‰ƒCƒ“‚Å‚Ķ•K{ŒŸø‚Ę‚³‚ź‚Ä‚¢‚éB‚ķ‚Ŗ‘‚É‚Ø‚¢‚Ä‚ĶCAmp-CML TMA –@‚É‚ę‚éBCR-ABL1 ‚ĢŒŸø‚Ķ•ŪŒÆ“K—p‚Ę‚Č‚Į‚Ä‚¢‚é‚ŖC‘ŪŽw•W‚Å•ā³‚³‚ź‚½BCR-ABL1 ‚Ģ’č—ŹRT-PCR ŒŸø‚Ŗ•ŪŒÆf—Ć‚ÅŽ{s‚³‚ź‚邱‚Ę‚Ŗ‹‚ß‚ē‚ź‚éB

‹ļ‘Ģ“I‚ČTKI ‚ĢŽ”—ĆŒų‰Ź”»’č‚Ģƒ^ƒCƒ~ƒ“ƒO‚ĶˆČ‰ŗ‚Ģ’Ź‚č‚Å‚ ‚éB

  1. Ž”—ĆŠJŽn‘O‚ĶCŒŒŽZ‚ĘŒŒ‰t‘œCœ‘‚ĢõF‘ĢŒŸø‚šŽ{s‚µCPh —z«—¦‚Ę•t‰Į“IõF‘ĢˆŁķ‚Ģ—L–³‚šŠm”F‚·‚éB
  2. Ž”—ĆŠJŽn’¼Œć‚ĶCŒŒŽZ‚ĘŒŒ‰t‘œ‚š–ˆT`2 T–ˆ‚ÉŒŸõ‚·‚éB
  3. CHR “ž’BŒć‚ĶC––½ŒŒFISH ‚š­‚Č‚­‚Ę‚ą3 ƒJŒŽ–ˆ‚ÉŒŸõ‚µCFISH ‚ÅPh —z«—¦‚Ŗ5“ˆČ‰ŗ‚Ę‚Č‚ź‚ĪCœ‘‚ĢõF‘ĢŒŸø‚šŽ{s‚·‚éB
  4. œ‘ŒŸø‚Ķ3 ƒJŒŽ–ˆ‚ÉŽ{s‚µCCCyR ‚Ģ”»’č‚Ŗ‚Å‚«‚ź‚ĪCˆČŒć‚Ķ––½ŒŒ‰t’č—ŹRT-PCR ‚ÅMR ‚šŒŸõ‚·‚éB’č—ŹRT-PCR ‚Ģ’˜‚µ‚¢‘‰Į‚Ŗ‚ ‚čCMMR ‚Ģ‘rŽø‚š‹^‚¤ź‡‚ĶCœ‘ŒŸø‚šs‚¤B‚Ü‚½CBCR-ABL1 “_“Ė‘R•ĻˆŁ‰šĶi•ŪŒÆ“K—pŠOj‚ɉĮ‚¦CƒCƒ}ƒ`ƒjƒuŒŒ’†”Z“xi•ŪŒÆ“K—pC“Į’č–ņÜŠĒ——æj‚Ŗ–Ś•W’l‚É’B‚µ‚Ä‚¢‚é‚ĶŽ”—Ć•ūj‚šŒˆ‚ß‚éŽQl‚É‚Č‚éB
•\3-1@CML ‚É‘Ī‚·‚é1st line ‚Ģƒ`ƒƒVƒ“ƒLƒi[ƒ[‘jŠQ–ņŽ”—Ć‚ĢŒų‰ŹiEuropean LeukemiaNet 2013 ”N”Łj
•]‰æŽž“_ Œų‰Ź
ŽŠ“K‘tŒų
Optimal
—v’ˆÓ
Warning
•s¬Œ÷
Failure
Ž”—Ć‘O
iƒx[ƒXƒ‰ƒCƒ“j
Žw“E‚Č‚µ ‚ƒŠƒXƒNC
‚Ü‚½‚ĶCCA/Ph{
Žw“E‚Č‚µ
3 ƒ•ŒŽ BCR-ABL1…10“C
‚Ü‚½‚ĶPh{…35“
BCR-ABL1„10“C
‚Ü‚½‚ĶPh{36`95“
CHR ‚É–¢“ž’BC
‚Ü‚½‚ĶPh{ „95“
6 ƒ•ŒŽ BCR-ABL1…1 “
‚Ü‚½‚ĶPh{0“
BCR-ABL11`10“C
‚Ü‚½‚ĶPh{1`35“
BCR-ABL1„10“C
‚Ü‚½‚ĶPh{„35“
12 ƒ•ŒŽ BCR-ABL1…0.1“ BCR-ABL10.1`1“ BCR-ABL1„1“C
‚Ü‚½‚ĶPh{„0“iCCyR –¢“ž’Bj
‚»‚ĢŒćC
‚Ē‚ĢŽž“_‚Å‚ą
BCR-ABL1…0.1“ CCA/Ph-i-7 ‚Ü‚½‚Ķ7q-j CHR ‚Ģ‘rŽøCCCyR ‚Ģ‘rŽøC
Šm’肵‚½MMR ‘rŽø–C
ABL ƒLƒi[ƒ[ƒhƒƒCƒ“‚Ģ•ĻˆŁC
CCA/Ph {

BCR-ABL1 ‚ĶBCR-ABL1IS ‚Å•\‚µ‚½’l
MMR ‚ĶBCR-ABL … 0.1“‚Å‚ ‚čMR3.0 ‚ ‚é‚¢‚Ķ‚»‚źˆČć‚ĢŒų‰Ź
–˜A‘±‚µ‚½2 ‰ń‚ĢBCR-ABL1≧1“
CCA/Ph {FPh õF‘Ģ‚Ģ•t‰Į“IõF‘ĢˆŁķCCCA/Ph-FPh õF‘ĢˆČŠO‚Ģ•t‰Į“IõF‘ĢˆŁķ

•\3-2 CML ‚É‘Ī‚·‚éƒCƒ}ƒ`ƒjƒuŽ”—ĆŽø”sŠ³ŽŅ‚É‘Ī‚·‚é2nd line ‚ĢTKI ‘jŠQ–ņŽ”—Ć‚ĢŒų‰Ź
iEuropean LeukemiaNet 2013 ”N”Łj
•]‰æŽž“_ Œų‰Ź
ŽŠ“K‘tŒų
Optimal
—v’ˆÓ
Warning
•s¬Œ÷
Failure
Ž”—Ć‘O
iƒx[ƒXƒ‰ƒCƒ“j
Žw“E‚Č‚µ ƒCƒ}ƒ`ƒjƒuŽ”—Ć‚É‚ÄCHR –¢’B¬
‚āCHR ‚Ģ‘rŽøC‰‰ńTKI Ž”—Ć‚É‚Ä
CyR –¢“ž’BC‚Ü‚½‚Ķ‚ƒŠƒXƒN
Žw“E‚Č‚µ
3 ƒ•ŒŽ BCR-ABL1…10“C
‚Ü‚½‚ĶPh{ƒ65“
BCR-ABL1„10“C
‚Ü‚½‚ĶPh{65`95“
CHR ‚É–¢“ž’BC
‚Ü‚½‚ĶPh{„95“C
‚Ü‚½‚ĶABL ƒLƒi[ƒ[ƒhƒƒCƒ“‚Ģ•ĻˆŁ
6 ƒ•ŒŽ <BCR-ABL1…10“
‚Ü‚½‚ĶPh{ƒ35“
BCR-ABL11`10“C
‚Ü‚½‚ĶPh{35`65“
BCR-ABL1„10“C
‚Ü‚½‚ĶPh{„35“
ABL ƒLƒi[ƒ[ƒhƒƒCƒ“‚Ģ•ĻˆŁ
12 ƒ•ŒŽ BCR-ABL1…1 “C
‚Ü‚½‚ĶPh{ 0“
BCR-ABL10.1`1 “C
‚Ü‚½‚ĶPh{1`35“
BCR-ABL1„10“C
‚Ü‚½‚ĶPh{„35“C
‚Ü‚½‚ĶABL ƒLƒi[ƒ[ƒhƒƒCƒ“‚Ģ•ĻˆŁ
‚»‚ĢŒćC
‚Ē‚ĢŽž“_‚Å‚ą
BCR-ABL1 … 0.1“ CCA/Ph-i-7 ‚Ü‚½‚Ķ7q-jC
‚Ü‚½‚ĶBCR-ABL1 „ 0.1“
CHR ‚Ģ‘rŽøCCCyR ‚Ģ‘rŽøC
Šm’肵‚½MMR ‘rŽø–C
ABL ƒLƒi[ƒ[ƒhƒƒCƒ“‚Ģ•ĻˆŁC
CCA/Ph

BCR-ABL1 ‚ĶBCR-ABL1IS ‚Å•\‚µ‚½’l
MMR ‚ĶBCR-ABL … 0.1“‚Å‚ ‚čMR3.0 ‚ ‚é‚¢‚Ķ‚»‚źˆČć‚ĢŒų‰Ź
–˜A‘±‚µ‚½2 ‰ń‚ĢMMR ‘rŽøiBCR-ABL1„0.1“j‚ŁC‚»‚Ģ‚¤‚æ1 ‚Ā‚ĶBCR-ABL1†1“
CCA/Ph {FPh õF‘Ģ‚Ģ•t‰Į“IõF‘ĢˆŁķCCCA/Ph-FPh õF‘ĢˆČŠO‚Ģ•t‰Į“IõF‘ĢˆŁķ

2DPh ‰A«‚Ģœ‘‘B«Žīį‡imyeloproliferative neoplasmsFMPNj

Ph ‰A«MPN ‚Ģ”­Ē‚ʐis‚É‚ąŽ¾Š³ŠÖ˜Aˆā“`Žqi‘½‚­‚Ŗ×–E“ą‚ ‚é‚¢‚Ķ×–E–Œ‚Ģƒ`ƒƒVƒ“ƒLƒi[ƒ[‚šƒR[ƒh‚·‚éj‚ĢˆŁķ‚Ŗ‘å‚«‚­ŠÖ—^‚µ‚Ä‚¢‚éB“Į‚ɁCPV ‚Ģ‚Ł‚Ś‘S—į‚ĘET ‚Ø‚ę‚ŃPMF ‚Ģ–ń”¼”‚ŖJAK2 ˆā“`Žq•ĻˆŁiV617F •ĻˆŁj‚š—L‚µCJAK2 ‚ĢPķ“I‚ČŠˆ«‰»‚Ŗ”­Ē‚ÉŠÖ—^‚µ‚Ä‚¢‚é‚ʍl‚¦‚ē‚ź‚éBˆź•ūCCEL/HES ‚Ģˆź•”‚ŖFIP1L1-PDGFRƒæ —Z‡ˆā“`Žq‚š—L‚µCmastocytosis ‚Ŗc-KIT ‚Ģ“_“Ė‘R•ĻˆŁ‚š—L‚µ‚Ä‚¢‚é1j2j17jB

PV ‚ĘET ‚Å‚Ķd“Ä‚ČŒŒšĒ‚Ģ‡•¹‚Ŗ—\Œć‚ɉe‹æ‚·‚邽‚߁CŒŒšĒ‚ĢHigh ƒŠƒXƒNŒQ‚šŒ©‹É‚ß‚é•K—v‚Ŗ‚ ‚éBˆź•ūCPMF ‚Å‚Ķ‹}«Šś‚Ö‚Ģi“W‚Ŗ—\Œć‚ɉe‹æ‚·‚邽‚߁C‚³‚Ü‚“‚Ü‚Č—\ŒćƒŠƒXƒN•Ŗ—Ž‚ŖŽŽ‚Ż‚ē‚ź‚Ä‚¢‚éB

3D^«ŌŒŒ‹…‘‰ĮĒ‚Ü‚½‚Ķ^«‘½ŒŒĒipolycythemia veraFPVj

1jPV ‚Ģ—\Œć•Ŗ—Ž18j

PV ‚Ģ¶–½—\Œć‚Ķ—ĒD‚Å‚ ‚čCŽ”—Ć‚É‚ę‚č10 ”NˆČć‚Ģ¶‘¶ŠśŠŌ’†‰›’l‚ŖŠś‘Ņ‚Å‚«‚éB‚»‚Ģ‚½‚߁C‡•¹‚·‚錌šĒ‚Ģ—\–h‚ŖŽ”—Ć‚ĢŽåŠį‚Ę‚Č‚éB”N—ī60 ĪˆČć‚Ü‚½‚ĶŒŒšĒ‚ĢŠł‰—š‚Ŗ‚ ‚銳ŽŅ‚ĶCŒŒšĒ‚ĢHigh ƒŠƒXƒNŠ³ŽŅ‚Å‚ ‚éi•\4jB

2jPV ‚ĢŽ”—ĆŠT—Ŗ
  1. ąbŒŒ—Ć–@FHt ’l45“–¢–ž‚š–Ś•W‚ɁCŒŒˆ³C–¬”‚Č‚Ē‚ĢzŠĀ“®‘Ō‚š‚Ż‚Č‚Ŗ‚ē1 ‰ń200`400 mL ‚ĢąbŒŒ‚šŒŽ‚É1`2 “x‚Ģƒy[ƒX‚ōs‚¤B‚—īŽŅ‚āSŒŒŠĒįŠQ‚š—L‚·‚é—į‚Å‚ĶCzŠĀ“®‘Ō‚Ģ‹}Œƒ‚Č•Ļ‰»‚Ŗ‚Č‚¢‚ꂤ‚ɁC­—Źi100`200 mLjC•p‰ń‚ĢąbŒŒ‚Ŗ–]‚Ü‚µ‚¢B
  2. ’į—p—ŹƒAƒXƒsƒŠƒ“—Ć–@FoŒŒ‚āĮ‰»ŠķĒó‚Č‚Ē‚Ģ‹ÖŠõ‚Å‚Č‚Æ‚ź‚ĪC75`100 mg/“ś‚ĢƒAƒXƒsƒŠƒ“‚ĢŒoŒū“Š—^‚Ŗ‘I‘š‚³‚ź‚éB
  3. HU —Ć–@FŒŒšĒ‚ĢHigh ƒŠƒXƒNŒQ‚Å‚ĶCąbŒŒ—Ć–@‚ɉĮ‚¦‚čœ‘—}§–ņ‚Å‚ ‚éHU —Ć–@‚š•¹—p‚·‚éBi‚½‚¾‚µC’·Šś“Š—^‚É‚ę‚é“ńŽŸ”­‚Ŗ‚ń‚ĢƒŠƒXƒN‚ŖŠ®‘S‚É‚Ķ”Ū’č‚³‚ź‚Ä‚¢‚Č‚¢‚½‚߁C“Į‚ÉŽį”NŽŅ‚Å‚ĶC—\ŒćˆöŽq‚ÉŠī‚Ć‚«Ē—į‚š‘I‘š‚·‚éj
  4. IFNƒæ—Ć–@F”DP’†‚ā‹“Ž™Šó–]ŽŅ‚Å‚ĶCĆŠļŒ`«‚Ģ–ā‘č‚©‚ēHU ‚É‘ć‚ķ‚č‘I‘š‚³‚ź‚éB
  5. ‚ŒŒˆ³C‚Ž‰ŒŒĒC”ģ–žC“œ”A•a‚Č‚Ē‚ĢC‚¢‚ķ‚ä‚錌šĒ‚Ģˆź”Ź“I‚ČƒŠƒXƒNƒtƒ@ƒNƒ^[‚Ŗ‚ ‚鏼‡‚ĶC‚±‚ź‚ē‚ĢŽ”—Ć‚šs‚¤B
  6. PV Œćœ‘üˆŪĒimyelofibrosisFMFjFPMF ‚ɏ€‚¶‚½Ž”—Ć–@‚š‘I‘š‚·‚éB
  7. ‹}«œ‘«”’ŒŒ•aiAMLj‚ ‚é‚¢‚Ķœ‘ˆŁŒ`¬ĒŒóŒQiMDSj‚Ö‚Ģi“WF‘±”­«‚ĢAML/MDS ‚É‘Ī‚µ‚Ä‚ĶCAML/MDS ‚ĢŽ”—Ć‚šs‚¤B
•\4@PV ‚ɂ؂Ƃ錌šĒ‚ĢƒŠƒXƒN•Ŗ—Ž
•ńŽŅ —\ŒćˆöŽq ƒŠƒXƒN•Ŗ—Ž
Tefferi et al.
iSemin Hematol. 2005 : 42 : 206j
”N—īƒ60 Ī
ŒŒšĒ‚ĢŠł‰‚Č‚µ
ŒŒ¬”Ā”ƒ150 –œ/ƒŹL
SŒŒŠĒ•a•Ļ‚ĢŠėŒÆˆöŽqi‹i‰ŒC‚ŒŒˆ³C‚¤‚ĮŒŒ«S•s‘S‚Ŗ‚Č‚¢j
‚Ģ‚·‚ׂĂĢ€–Ś‚š–ž‚½‚·
Low ƒŠƒXƒNŒQ
Low ƒŠƒXƒNŒQ‚É‚ąHigh ƒŠƒXƒNŒQ‚É‚ą‘®‚³‚Č‚¢ Intermediate ƒŠƒXƒNŒQ
”N—ī† 60 ĪC‚Ü‚½‚ĶŒŒšĒ‚ĢŠł‰‚Ŗ‚ ‚é High ƒŠƒXƒNŒQ

4D–{‘Ō«ŒŒ¬”ĀŒŒĒiessential thrombocythemiaFETj

1jET ‚Ģ—\Œć•Ŗ—Ž

ET ‚Ģ¶–½—\Œć‚Ķ—ĒD‚Å‚ ‚čCŒ’ķŽŅ‚Ę‚Ł‚Ś“Æ“™‚Ģ¶–½—\Œć‚ŖŠś‘Ņ‚³‚ź‚éB‚»‚Ģ‚½‚߁C‡•¹‚·‚錌šĒ‚Ģ—\–h‚ŖŽ”—Ć‚ĢŽåŠį‚Ę‚Č‚éB”N—ī60 ĪˆČć‚Ü‚½‚ĶŒŒšĒ‚ĢŠł‰—š‚Ŗ‚ ‚銳ŽŅ‚ĶCŒŒšĒ‚ĢHigh ƒŠƒXƒNŠ³ŽŅ‚Å‚ ‚éB

ŒŒ¬”Ā”‘‰Į‚ā‚ŒŒˆ³C‚Ž‰ŒŒĒC“œ”A•aC‹i‰Œ‚Č‚Ē‚šŒŒšĒ‚ĢŠėŒÆˆöŽq‚Ę‚µ‚Ĉµ‚¤‚©‚Ķ•ń‚É‚ę‚čˆŁ‚Č‚Į‚Ä‚Ø‚čCŒ‹˜_‚Ķ“¾‚ē‚ź‚Ä‚¢‚Č‚¢BÅ‹ßC”’ŒŒ‹…”‚āJAK2 •ĻˆŁ‚ĢŠ„‡‚Ŗ‘½‚¢‚ĘŒŒšĒ‚Ŗ¶‚¶‚ā‚·‚¢‚Ę‚Ģ•ń‚ą‚ ‚éBˆź”Ź“I‚É‚ĶC”N—īCŒŒšĒ‚ĢŠł‰CŒŒ¬”Ā”‚ę‚čCLow ƒŠƒXƒN‚ĘHigh ƒŠƒXƒN‚É•Ŗ—Ž‚³‚ź‚Ä‚¢‚éi•\5j19jBÅ‹ßC”N—ī‚ʏ‰fŽž”’ŒŒ‹…”‚ę‚čC¶–½—\Œć‚ÉŠÖ‚µ‚Ä3 ‚Ā‚ĢƒŠƒXƒN‚É•Ŗ—Ž‚·‚é20j‚±‚Ę‚Ŗ’ń„‚³‚ź‚Ä‚¢‚éi•\6jB

2jET ‚ĢŽ”—ĆŠT—Ŗ
  1. ŒŒšĒLow ƒŠƒXƒN‚ĢŽ”—ƁF’芜“I‚ČŒo‰ßŠĻŽ@‚Ģ‚Ż‚šs‚¤Bœ‘—}§‚š‚«‚½‚·–ņÜ‚āŒŒ¬”Ā‚š’įŒø‚·‚é–ņÜ‚Ģ“Š—^‚Ķ•s—v‚Å‚ ‚é19jB
  2. ŒŒšĒHigh ƒŠƒXƒN‚ĢŽ”—ƁF‡•¹‚·‚錌šĒ‚Ģ—\–h‚š–Ś“I‚Ę‚µ‚ÄHU ‚Ę’į—p—ŹƒAƒXƒsƒŠƒ“‚š•¹—p‚·‚é21j22jBi‚½‚¾‚µC’·Šś“Š—^‚É‚ę‚é“ńŽŸ”­‚Ŗ‚ń‚ĢƒŠƒXƒN‚ŖŒœ”O‚³‚ź‚邽‚߁C“Į‚ÉŽį”NŽŅ‚Å‚ĶC—\ŒćˆöŽq‚ÉŠī‚Ć‚«Ē—į‚š‘I‘š‚·‚éBjŒŒ¬”Ā”‚Ŗ’˜‘‚µ‚Ä‚¢‚鏼‡iŒŒ¬”Ā”150 –œ/ƒŹL ˆČćj‚ĢƒAƒXƒsƒŠƒ“’P“Ę“Š—^‚ĶCoŒŒ‚š•’·‚·‚éŠėŒÆ‚Ŗ‚ ‚邽‚߁CHU “Š—^‚É‚ę‚čŒŒ¬”Ā”‚šŒø‚ē‚µ‚Ä‚©‚ē“Š—^‚·‚éBET ‚Ģ”­Ē”N—ī‚ĶPV ‚Ę”ä‚׎į‚­C‚ā‚ā—«‚ɍD”­‚·‚邱‚Ę‚©‚ēC”DPC‹“Ž™Šó–]‚Ŗ–ā‘č‚ʂȂ邱‚Ę‚Ŗ‚ ‚éB‚±‚Ģ‚ꂤ‚ȏź‡‚ĶCHU ‚É‘ć‚ķ‚čCIFNƒæ‚š“Š—^‚·‚éBHU •s‘Ļ—e‚ą‚µ‚­‚Ķ’ļR«‚ĢĒ—į‚É‚ĶC‚ķ‚Ŗ‘‚Å‚Ķ•ŪŒÆ“K—p‚Ŗ‚Č‚¢‚ŖC‰¢•Ä‚Å‚ĶƒAƒiƒOƒŒƒ‰ƒCƒh‚ŖŽg—p‚³‚ź‚Ä‚¢‚éBƒAƒiƒOƒŒƒ‰ƒCƒhi{’į—p—ŹƒAƒXƒsƒŠƒ“j‚ĶHUi{’į—p—ŹƒAƒXƒsƒŠƒ“j‚ę‚čĆ–¬ŒŒšĒ‚ĢƒŠƒXƒN‚Ķ’į‚¢‚ŖCS–[ŒŒšCd“Ä‚ČoŒŒCœ‘üˆŪĒ‚Ö‚Ģi“W•p“x‚Ŗ‚‚¢22jB
y‘ę1.1”ŏC³z
  1. ŒŒšĒHigh ƒŠƒXƒN‚ĢŽ”—ƁF‡•¹‚·‚錌šĒ‚Ģ—\–h‚š–Ś“I‚Ę‚µ‚ÄHU ‚Ę’į—p—ŹƒAƒXƒsƒŠƒ“‚š•¹—p‚·‚é21j22jBi‚½‚¾‚µC’·Šś“Š—^‚É‚ę‚é“ńŽŸ”­‚Ŗ‚ń‚ĢƒŠƒXƒN‚ŖŒœ”O‚³‚ź‚邽‚߁C“Į‚ÉŽį”NŽŅ‚Å‚ĶC—\ŒćˆöŽq‚ÉŠī‚Ć‚«Ē—į‚š‘I‘š‚·‚éBjŒŒ¬”Ā”‚Ŗ’˜‘‚µ‚Ä‚¢ ‚鏼‡iŒŒ¬”Ā”150 –œ/ƒŹLˆČćj‚ĢƒAƒXƒsƒŠƒ“’P“Ę“Š—^‚ĶCoŒŒ‚š•’·‚·‚éŠėŒÆ‚Ŗ‚ ‚邽‚߁CHU “Š—^‚É‚ę‚čŒŒ¬”Ā”‚šŒø‚ē‚µ‚Ä‚©‚ē“Š—^‚·‚éBET ‚Ģ”­Ē”N—ī‚ĶPV ‚Ę”ä‚׎į‚­C‚ā‚ā—«‚ɍD”­‚·‚邱‚Ę‚©‚ēC”DPC‹“Ž™Šó–]‚Ŗ–ā‘č‚ʂȂ邱‚Ę‚Ŗ‚ ‚éB‚±‚Ģ‚ꂤ‚ȏź‡‚ĶCHU ‚É‘ć‚ķ‚čCIFNƒæ‚š“Š—^‚·‚éBHU •s‘Ļ—e‚ą‚µ‚­‚Ķ’ļR«‚ĢĒ—į‚É‚ĶAƒAƒiƒOƒŒƒŠƒhi{ƒAƒXƒsƒŠƒ“j‚š“Š—^‚·‚éBƒAƒiƒOƒŒƒŠƒh+ ’į—p—ŹƒAƒXƒsƒŠƒ“‚ĶAHU+ ’į—p—ŹƒAƒXƒsƒŠƒ“‚ę‚čĆ–¬ŒŒšĒ‚ĢƒŠƒXƒN‚Ķ’į‚¢‚ŖAS–[ŒŒšAd“Ä‚ČoŒŒAœ‘üˆŪĒ‚Ö‚Ģi“W•p“x‚Ŗ‚‚­AEFS ‚Ķ—ņ‚é‚Ę‚¢‚¤•ń22j‚ʁAƒAƒiƒOƒŒƒŠƒh‚ĶHU ‚ĘŠr‚×EFS ‚É—LˆÓ·‚š”F‚ß‚Č‚¢33j‚Ę‚Ģ•ń‚Ŗ‚ ‚éB
•\5@ET ‚ɂ؂Ƃ錌šĒ‚ĢƒŠƒXƒN•Ŗ—Ž
—\ŒćˆöŽq ƒŠƒXƒN•Ŗ—Ž
”N—īƒ60 ĪC‚©‚ĀŒŒšĒ‚ĢŠł‰‚Ķ‚Č‚µC
‚©‚ĀŒŒ¬”Ā”ƒ150 –œ/ƒŹL
Low ƒŠƒXƒNŒQ
”N—ī†60 ĪC‚Ü‚½‚ĶŒŒšĒ‚ĢŠł‰‚ ‚čC
‚Ü‚½‚ĶŒŒ¬”Ā”†150 –œ/ƒŹL
High ƒŠƒXƒNŒQ
•\6@ET ‚ɂ؂Ƃ鐶–½—\Œć‚ĢƒŠƒXƒN•Ŗ—Ž
•ńŽŅ —\ŒćˆöŽq ƒŠƒXƒN•Ŗ—Ž ¶‘¶ŠśŠŌ’†‰›’li”Nj
Wolanskyj et al.
iMayo Clin Proc. 2006 ; 81 : 159j
”N—īƒ60 ĪC
‚©‚Ā”’ŒŒ‹…”ƒ15,000/ƒŹL
Low ƒŠƒXƒNŒQ 25.3
”N—ī†60 ĪC
‚Ü‚½‚Ķ”’ŒŒ‹…”†15,000/ƒŹL
Intermediate ƒŠƒXƒNŒQ 16.9
”N—ī†60 ĪC
‚©‚Ā”’ŒŒ‹…”†15,000/ƒŹL
High ƒŠƒXƒNŒQ 10.3

5DŒ“”­«œ‘üˆŪĒiprimary myelofibrosisFPMFj

1jPMF ‚Ģ—\Œć•Ŗ—Ž

‚±‚ź‚܂ōL‚­—p‚¢‚ē‚ź‚Ä‚«‚½Lille •Ŗ—Ž‚Č‚Ē‚ɉĮ‚¦C3 ‚Ā‚Ģ‘Ū“I—\Œć•Ŗ—Žiinternational prognostic scoring systemFIPSSj‚Ŗ—Տ°‰ž—p‚³‚ź‚Ä‚¢‚éB”N—ī„65 ĪC‘ĢdŒø­C–éŠŌ“Š¾C”­”M‚Č‚Ē‚Ģ—Տ°ĒóCƒwƒ‚ƒOƒƒrƒ“’liHbjƒ10 g/dLCf’fŽžWBC„25,000/ƒŹLC––½ŒŒ‰t’†‚Ģ‰č‹…Š„‡† 1“‚Ģ5 ‚Ā‚š—\ŒćˆöŽq‚Ę‚·‚éIPSS 23jC‘Oq‚Ģ5 ˆöŽq‚ɈŁ‚Č‚Į‚½d‚Ż•t‚Æ‚š‚µ‚½Dynamic IPSSiDIPSSj24jCDIPSS ‚ɐõF‘ĢˆŁķCŒŒ¬”Ā”C—AŒŒˆĖ‘¶«‚š•t‰Į‚µ‚½DIPSS Plus 25j‚Ŗ’ń„‚³‚ź‚Ä‚¢‚éi•\7jBˆöŽq‚Ģ”‚É‚ę‚čCLowCIntermediate-1iInt-1jCIntermediate-2iInt-2j‚»‚µ‚ÄHigh ‚Ģ4 ‚Ā‚ĢƒŠƒXƒNƒOƒ‹[ƒv‚É•Ŗ—Ž‚³‚źC—\Œć—\‘Ŗ‚Ŗ‰Ā”\‚Å‚ ‚čCŽ”—Ć‘I‘š‚É—p‚¢‚ē‚ź‚éB

•\7@Œ“”­«œ‘üˆŪĒ‚Ģ—\Œć—\‘Ŗƒ‚ƒfƒ‹
—\Œć—\‘Ŗƒ‚ƒfƒ‹ —\Œć•s—ĒˆöŽqiƒXƒRƒAj —\Œć•]‰æ
ƒXƒRƒA
‚Ģ‡Œv
ƒŠƒXƒN•Ŗ—Ž ¶‘¶ŠśŠŌ
’†‰›’li”Nj
Lille •Ŗ—Ž
iBlood 1996 ; 88 : 1013j
Hbƒ10 g/dLi1j
WBCƒ4,000/ƒŹLi1j
‚Ü‚½‚Ķ
„30,000ƒŹLi1j
0
1
2
Low ƒŠƒXƒN
Intermediate ƒŠƒXƒN
High ƒŠƒXƒN
7.8
2.2
1.1
IPSS
iBlood 2009 ; 113 : 2895j
”N—ī„65 Īi1j
”­”ME–éŠŌ“Š¾E‘ĢdŒø­‚ĢŽ‘±i1j
Hbƒ10 g/dLi1j
WBC„25,000/ƒŹLi1j
––½ŒŒ ‰č‹…†1“i1j
0
1
2
† 3
Low ƒŠƒXƒN
Intermediate-1 ƒŠƒXƒN
Intermediate-2 ƒŠƒXƒN
High ƒŠƒXƒN
11.3
7.9
4.0
2.3
DIPSS/aaDIPSS
iBlood 2010 ; 115 : 1703j
DIPSSF
”N—ī„65 Īi1j
”­”ME–éŠŌ“Š¾E‘ĢdŒø­‚ĢŽ‘±i1j
Hbƒ10 g/dLi2j
WBC„25,000/ƒŹLi1j
––½ŒŒ ‰č‹…†1“i1j
0
1 `2
3 `4
5 `6
Low ƒŠƒXƒN
Intermediate-1 ƒŠƒXƒN
Intermediate-2 ƒŠƒXƒN
High ƒŠƒXƒN
“ž’B‚¹‚ø
14.2
4.0
1.5
Age-adjusted DIPSSi65 Ī–¢–žjF
”­”ME–éŠŌ“Š¾E‘ĢdŒø­‚ĢˆŪŽi2j
Hbƒ10 g/dLi2j
WBC„25,000/ƒŹLi1j
––½ŒŒ ‰č‹…†1“i2j
0
1 `2
3 `4
„ 4
Low ƒŠƒXƒN
Intermediate-1 ƒŠƒXƒN
Intermediate-2 ƒŠƒXƒN
High ƒŠƒXƒN
“ž’B‚¹‚ø
9.8
4.8
2.3
DIPSS plus
iJ Clin Oncol 2011 ; 29 : 392j
—\Œć•s—ĒŠjŒ^
i•”ŽGŠjŒ^i3 Žķ—ŽˆČć‚ĢˆŁķjC+8C
-7/7q-Cii17qjC-5/5q-C12p-Cinv (3jC
11q23 ˆŁķji1j
ŒŒ¬”Āƒ100,000/ƒŹLi1j
—AŒŒ‚Ģ•K—v«i1j
DIPSS Intermediate-1 ƒŠƒXƒNi1j
DIPSS Intermediate-2 ƒŠƒXƒNi2j
DIPSS High ƒŠƒXƒNi3j
0
1
2 `3
4 `6
Low ƒŠƒXƒN
Intermediate-1 ƒŠƒXƒN
Intermediate-2 ƒŠƒXƒN
High ƒŠƒXƒN
15.4
6.5
2.9
1.3
2jPMF ‚ĢŽ”—ĆŠT—Ŗ
  1. Low ‚Ø‚ę‚ŃInt-1 ƒŠƒXƒN‚ĢŽ”—ƁF—Տ°ĒóC•nŒŒĒó‚šŒ‡‚­Š³ŽŅ‚Ģ¶‘¶ŠśŠŌ‚Ķ10 ”N‚š’“‚¦‚邽‚߁CŒ»Žž“_‚Å‚ĶŒo‰ßŠĻŽ@‚Ŗ–]‚Ü‚µ‚¢B
  2. Int-2 ‚Ø‚ę‚ŃHigh ƒŠƒXƒN‚ĢŽ”—ƁF“K‰ž‰Ā”\”N—ī‚Å‚ ‚ź‚ĪCallo-HSCT ‚ĶŖŽ”“IŽ”—Ć–@‚Å‚ ‚čCf’f‘Šś‚ę‚萄§‚³‚ź‚éBallo-HSCT ‚Ģ“K‰ž‚Ę‚Č‚ē‚Č‚¢Š³ŽŅ‚É‘Ī‚µ‚Ä‚ĶC—Տ°Ēó‚ĢŒyŒø‚šŽå–Ś“I‚Ę‚·‚é‘ĪĒ—Ć–@‚ŖŽ{s‚³‚ź‚éB
  3. –ņ•Ø—Ć–@Fƒ^ƒ“ƒpƒN“Ɖ»ƒXƒeƒƒCƒhƒzƒ‹ƒ‚ƒ“–ņCHUC•›t”ēŽæƒXƒeƒƒCƒhC‰¢•Ä‚Å‚ĶCƒTƒŠƒhƒ}ƒCƒhiTHALjCƒŒƒiƒŠƒhƒ~ƒhiLENjCJAK ‘jŠQÜiruxolitinibj26j‚Č‚Ē‚Ŗ—p‚¢‚ē‚ź‚Ä‚¢‚éB‚ķ‚Ŗ‘‚Å‚ąJAK ‘jŠQÜ‚Ģ—Տ°ŽŽŒ±‚Ŗs‚ķ‚ź‚Ä‚Ø‚čC•ŪŒÆ“K—p‚Ę‚Č‚ź‚ĪCŽg—p‰Ā”\‚Å‚ ‚éB
y‘ę1.1”ŏC³z
  1. –ņ•Ø—Ć–@Fƒ^ƒ“ƒpƒN“Ɖ»ƒXƒeƒƒCƒhƒzƒ‹ƒ‚ƒ“–ņAHUA•›t”ēŽæƒXƒeƒƒCƒhA‰¢•Ä‚Å‚ĶAƒTƒŠƒhƒ}ƒCƒhiTHALjAƒŒƒiƒŠƒhƒ~ƒhiLENjAJAK ‘jŠQÜiƒ‹ƒLƒ\ƒŠƒ`ƒjƒuj26j‚Č‚Ē‚Ŗ—p‚¢‚ē‚ź‚Ä‚¢‚éB‚ķ‚Ŗ‘‚Å‚ąƒ‹ƒLƒ\ƒŠƒ`ƒjƒu‚Ŗ•ŪŒÆ“K—p‚Ę‚Č‚čA—Տ°‰ž—p‚Ŗ‰Ā”\‚Ę‚Č‚Į‚½B
  1. äB‘Ÿ“EopEäBĘŽĖF‹‘åäBŽī‚É‚ę‚éįu’ɁC•nŒŒCŒŒ‹…Œø­Cd“x‚Ģ–å–¬ˆ³˜“i‚É‘Ī‚µ‚ÄäB‘Ÿ“Eop‚āäBĘŽĖ‚ŖŽ{s‚³‚ź‚éB
  2. ‹}«”’ŒŒ•a‚Ö‚Ģi“WF‹}«”’ŒŒ•a‚ɏ€‚¶‚½Ž”—Ć‚šŽ{s‚·‚éB

6D‚»‚Ģ‘¼‚ĢMPN ‚Ģ•a‘Ō‚ĘŽ”—Ć‚É‚Ā‚¢‚Ä

ć‹LˆČŠO‚ĢMPN ‚ŁC”äŠr“I‹H‚ČŽ¾Š³‚Å‚ ‚閝«D’†‹…«”’ŒŒ•aichronic neutrophilic leukemiaF CNLj27jC–«DŽ_‹…«”’ŒŒ•aichronic eosinophilic leukemiaFCELj28jC”ģ–ž×–EĒimastocytosisj 29jC•Ŗ—Ž•s”\œ‘‘B«Žīį‡imyeloproliferative neoplasmsCunclassifiableFMPN Uj30j‚É‚Ā‚¢‚Ä’Ē‰Į‚·‚éB

  1. CNL ‚Ķ¬n‚µ‚½D’†‹…‚Ģ’˜‚µ‚¢‘‰Į‚š‚Ż‚ʂ߁CBCR-ABL1 —Z‡ˆā“`Žq‚š”F‚ß‚Č‚¢‚±‚Ę‚Ŗf’f‹K€‚Ę‚Č‚Į‚Ä‚¢‚éBˆź”Ź“I‚É‚Ķ•nŒŒCŒŒ¬”ĀŒø­‚š”ŗ‚¢ŠÉ™‚Ȑis‚šŽ¦‚·‚ŖC¶–½—\Œć‚Ķ6 ƒJŒŽ‚©‚ē20 ”N‚Ę•‚ŖL‚¢BĒóŠÉ˜a“I‚ČŽ”—Ć‚Ŗ’†S‚Ę‚Č‚éBŽž‚É‹}«”’ŒŒ•a‰»‚š‚«‚½‚·‚ŖC‰»Šw—Ć–@‚ÉŠÖ˜A‚µ‚½“ńŽŸ‚Ŗ‚ń‚Ę‚ĢŠÖŒW‚Ķ•s–¾‚Å‚ ‚éB
  2. CEL ‚ĶDŽ_‹…‘O‹ģ×–E‚ĢƒNƒ[ƒiƒ‹‚Č‘B‚ĢŒ‹‰ŹC––½ŒŒ‚É‚Ä’˜‚µ‚¢DŽ_‹…‘‘½Ē‚š”F‚ß‚éMPN ‚Å‚ ‚éBDŽ_‹…‘‰ĮĒŒóŒQihypereosinphilic syndromeFHESj‚Ģœ‘‘Bvariant (HES/CEL) ‚Ę‘Ø‚¦‚ē‚źC–³Ēó‚ĢĒ—į‚©‚ēC‘ŸŠķZ‚š”ŗ‚¢‚³‚Ü‚“‚Ü‚Č—Õ°Ēó‚š”ŗ‚¢C¶–½—\Œć‚ą‹É‚ß‚Ä•‚ŖL‚¢BHES ‚Ģ10`14“‚ÅFIP1L1-PDGFƒæ —Z‡ˆā“`Žq‚ŖŒŸo‚³‚źCƒNƒ[ƒiƒŠƒeƒB‚ŖŲ–¾‚³‚ź‚éB‚±‚¤‚µ‚½HES/CEL ‚Å‚ĶCFIP1L1-PDGFRƒæ ƒ`ƒƒVƒ“ƒLƒi[ƒ[‚š‘jŠQ‚·‚éƒCƒ}ƒ`ƒjƒu‚Ģ—LŒų«‚Ŗ”F‚ß‚ē‚ź‚é31jB
  3. Mastocytosis ‚Ķ”ģ–ž×–Eimast cellj‚ĢŽīį‡«‘B‚É‚ę‚čˆų‚«‹N‚±‚³‚ź‚éMPN ‚Å‚ ‚čC–«‚¶‚ń‚Ü‚µ‚ń—l‚Ģ”ē•†”ģ–ž×–EĒ‚©‚ē‘Sg‘ŸŠķ‚ɐZ‚·‚é”ģ–ž×–EĒC”ģ–ž×–E”’ŒŒ•a‚Č‚Ē‚É•Ŗ—Ž‚³‚ź‚»‚ĢĒóC—\Œć‚ą‹É‚ß‚Ä‘½Ź‚Å‚ ‚éB”ģ–ž×–EĒ‚Å‚Ķ”ģ–ž×–E‚Ģ‘B‚ÉŠÖ—^‚µ‚Ä‚¢‚éc-KIT ‚Ģ“_“Ė‘R•ĻˆŁ‚Ŗ•ń‚³‚ź‚Ä‚¢‚é29jB
  4. •Ŗ—Ž•s”\Œ^MPN ‚Ķć‹L‚ĢMPN ‚Ģf’f‹K€‚ÉŠ®‘S‚ɍ‡’v‚µ‚Č‚¢‚ą‚Ģ‚ĢC2 ‚ĀˆČć‚ĢMPN ‚Ģ•aó‚ŖƒI[ƒo[ƒ‰ƒbƒv‚µ‚Ä‚¢‚鎾Š³ŠT”O‚Å‚ ‚éBPV/ET/PMF ‚Ģ‹É‚ß‚Ä”­Ē‘Šś‚ĢĒ—į‚©C‹t‚ɐis‚µ‚½ó‘Ō‚ĢĒ—į‚Ģ‚±‚Ę‚Ŗ‘½‚¢30jB
yŽQl•¶Œ£z

1j Vardiman JW, et al. Introduction and overview of the classification of the myeloid neoplasms. Swerdlow SH, et al. eds. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon, IARC ; 2008 : pp18-30.iƒŒƒrƒ…[j

2j Vardiman JW, et al. Chronic myelogenous leukaemia, BCR-ABL1 positive. Swerdlow SH, et al. eds. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon, IARC ; 2008 : pp32-7.iƒŒƒrƒ…[j

3j Sokal JE, et al. Prognostic discrimination ing good-riskh chronic granulocytic leukemia. Blood. 1984 ; 63i4j: 789-99.i3iAj

4j Hasford J, et al. A new prognostic score for survival of patients with chronic myeloid leukemia treated with interferon alfa. Writing Committee for the Collaborative CML Prognostic Factors Project Group. J Natl Cancer Inst. 1998 ; 90i11j : 850-8.i3iAj

5j Hasford J, et al. Predicting complete cytogenetic response and subsequent progression-free survival in 2060 patients with CML on imatinib treatment : the EUTOS score. Blood. 2011 ; 118i3j : 686-92.i3iAj

6j Baccarani M, et al. Chronic myeloid leukemia : an update of concepts and management recommendations of European LeukemiaNet. J Clin Oncol. 2009 ; 27i35j : 6041-51.iƒŒƒrƒ…[j

7j Baccarani M, et al. European LeukemiaNet recommendations for the management of chronic myeloid leukemia : 2013. Blood. 2013 ; 122i6j : 872-84.iƒŒƒrƒ…[j

8j OfBrien SG, et al. Imatinib compared with interferon and low-dose cytarabine for newly diagnosed chronic- phase chronic myeloid leukemia. N Engl J Med. 2003 ; 348i11j : 994-1004.i1iiDivj

9j Druker BJ, et al. Five-year follow-up of patients receiving imatinib for chronic myeloid leukemia. N Engl J Med 2006 ; 355i23j : 2408-17.i2Diiij

10j Kantarjian HM, et al. Survival benefit with imatinib mesylate versus interferon-alpha-based regimens in newly diagnosed chronic-phase chronic myelogenous leukemia. Blood. 2006 ; 108i6j : 1835-40.i3iiAj

11j Ohnishi K, et al. Long-term outcome following imatinib therapy for chronic myelogenous leukemia, with assessment of dosage and blood levels : the JALSG CML202 study. Cancer Sci. 2012 ; 103 i6j : 1071-8.i3iiiAj

12j Saglio G, et al. Nilotinib versus imatinib for newly diagnosed chronic myeloid leukemia. N Engl J Med. 2010 ; 362i24j : 2251-9.i1iiDivj

13j Kantarjian H, et al. Dasatinib versus imatinib in newly diagnosed chronic-phase chronic myeloid leukemia. N Engl J Med. 2010 ; 362i24j : 2260-70.i1iiDivj

14j Biggs JC, et al. Treatment of chronic myeloid leukemia with allogeneic bone marrow transplantation after preparation with BuCy2. Blood. 1992 ; 80i5j : 1352-7.i3iiiAj

15j Hehlmann R, et al. Randomized comparison of interferon-alpha with busulfan and hydroxyurea in chronic myelogenous leukemia. The German CML Study Group. Blood. 1994 ; 84i12j : 4064-77.i1iiAj

16j Cross NC, et al. Standardized definitions of molecular response in chronic myeloid leukemia. Leukemia. 2012 ; 26i10j : 2172-5.iƒŒƒrƒ…[j

17j Vardiman JW, et al. The 2008 revision of the World Health OrganizationiWHOj classification of myeloid neoplasms and acute leukemia : rationale and important changes. Blood. 2009 ; 114i5j : 937-51.iƒŒƒrƒ…[j

18j Tefferi A, et al. Polycythemia vera : scientific advances and current practice. Semin Hematol. 2005 ; 42i4j: 206-20.iƒŒƒrƒ…[j

19j Ruggeri M, et al. No treatment for low-risk thrombocythaemia : results from a prospective study. Br J Haematol. 1998 ; 103i3j : 772-7.i2Cj

20j Wolanskyj AP, et al. Essential thrombocythemia beyond the first decade : life expectancy, long-term complication rates, and prognostic factors. Mayo Clin Proc. 2006 ; 81i2j : 159-66.i3iiAj

21j Palandri F, et al. Long-term follow-up of 386 consecutive patients with essential thrombocythemia : safety of cytoreductive therapy. Am J Hematol. 2009 ; 84i4j : 215-20.i3iiAj

22j Harrison CN, et al. Hydroxyurea compared with anagrelide in high-risk essential thrombocythemia. N Engl J Med. 2005 ; 353i1j : 33-45.i1iiCj

23j Cervantes F, et al. New prognostic scoring system for primary myelofibrosis based on a study of the International Working Group for Myelofibrosis Research and Treatment. Blood. 2009 ; 113 i13j : 2895-901. i3iAj

24j Passamonti F, et al. A dynamic prognostic model to predict survival in primary myelofibrosis : a study by the IWG-MRT (International Working Group for Myeloproliferative Neoplasms Research and Treatment). Blood. 2010 ; 115i9j : 1703-8.i3iAj

25j Gangat N, et al. DIPSS plus : a refined Dynamic International Prognostic Scoring System for primary myelofibrosis that incorporates prognostic information from karyotype, platelet count, and transfusion status. J Clin Oncol. 2011 ; 29i4j : 392-7.i3iAj

26j Verstovsek S, et al. A double-blind, placebo-controlled trial of ruxolitinib for myelofibrosis. N Engl J Med. 2012 ; 366i9j : 799-807.i1iDivj

27j Bain BJ, et al. Chronic neutrophilic leukaemia. Swerdlow SH, Campo, et al. eds. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon, IARC ; 2008 : pp38-9.iƒŒƒrƒ…[j

28j Bain BJ, et al. Chronic eosinophilic leukaemia, not otherwise specified. Swerdlow SH, et al. eds. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon, IARC ; 2008 : pp51-3.iƒŒƒrƒ…[j

29j Horny H-P, et al. Mastocytosis. Swerdlow SH, et al. eds. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon, IARC ; 2008 : pp54-63.iƒŒƒrƒ…[j

30j Kvasnicka HM, et al. Myeloproliferative neoplasm, unclassifiable. Swerdlow SH, et al. eds. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon, IARC ; 2008 : pp64-5.iƒŒƒrƒ…[j

31j Cools J, et al. A tyrosine kinase created by fusion of the PDGFRA and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome. N Engl J Med. 2003 ; 348 i13j : 1201-14.i3iiiDivj

y‘ę1.1”Å’Ē‹Lz

32j Gambacorti-Passerini C, et al. Bosutinib efficacy and safety in chronic phase chronic myeloid leukemia after imatinib resistance or intolerance:minimum 24-month follow-up. Am J Hematol 2014: 89(7): 732-42.i2Diiij

33j Gisslinger H, et al. Anagrelide compared with hydroxyurea in WHO-classified essential thrombocythemia: the ANAHYDRET Study, a randomized controlled trial. Blood. 2013;121(10):1720-1728.i1iiDivj



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1DCML ‚ĢƒAƒ‹ƒSƒŠƒYƒ€

Œ»Ż‚ĢCML Ž”—Ć‚ĢKey Drug ‚ĶTKI ‚Å‚ ‚éBCML-CP Šś‚É‚ĶTKIiƒCƒ}ƒ`ƒjƒuCƒjƒƒ`ƒjƒuCƒ_ƒTƒ`ƒjƒuj‚š“Š—^‚·‚éiCQ1jBŽ”—ĆŠJŽnŒćCŽŠ“K‘tŒųiOptimalj‚Ģź‡‚ĶŽ”—ĆŒp‘±iCQ2jCWarningi—v’ˆÓj‚Ģź‡‚Ķƒ‚ƒjƒ^ƒŠƒ“ƒO‚š•p‰ń‚É‚µ‚āCFailurei•s¬Œ÷j‚Ģź‡‚ĶƒCƒ}ƒ`ƒjƒu‚Ķ‘¼‚ĢTKI ‚ցCƒjƒƒ`ƒjƒu‚Ķƒ_ƒTƒ`ƒjƒuCƒ_ƒTƒ`ƒjƒu‚Ķƒjƒƒ`ƒjƒu‚ÖŽ”—Ć•ĻX‚Ę“Æ–E‚ĢHLA ŒŸõ‚šs‚¤iCQ3jB“_“Ė‘R•ĻˆŁ‰šĶ‚ɉĮ‚¦CƒCƒ}ƒ`ƒjƒuŒŒ’†”Z“x‚Ŗ–Ś•W’l‚É’B‚µ‚Ä‚¢‚é‚©‚ĶŽ”—Ć•ūj‚šŒˆ‚ß‚éŽQl‚É‚Č‚éiCQ4jBCML-CP ‚©‚ēi“W‚µ‚½AP Šś‚É‚Ķ–¢Žg—pTKI ‚ÅŽ”—Ć‚µCBP Šś‚É‚ĶTKI ’P“Ę‚ą‚µ‚­‚Ķ‹}«”’ŒŒ•a‚ɏ€‚¶‚½‰»Šw—Ć–@‚š•¹—p‚·‚éiCQ5jBˆŚA“K‰ž‚Å‚ ‚ź‚ĪCallo-HSCT ‚š„§‚·‚éiCQ5jBTKI Ž”—Ć’†‚ÉT315I “_“Ė‘R•ĻˆŁ‚ŖŠm”F‚³‚ź‚½ź‡‚ĶCallo-HSCT ‚ą‚µ‚­‚ĶT315I ‚É—LŒų‚Č–ņÜ‚Ģ—Տ°ŽŽŒ±‚š‘I‘š‚·‚邱‚Ę‚Ŗl—¶‚³‚ź‚éBŒ»Ż‚Ģ‚Ę‚±‚ėTKI ‚š’†Ž~‚Å‚«‚é‹K€‚Ķ‚Č‚­C•ŖŽqˆā“`Šw“IŠ®‘S‘tŒųiCMRj‚Ŗ“¾‚ē‚ź‚Ä‚ąŽ”—Ć‚šŒp‘±‚·‚ׂ«‚Å‚ ‚éiCQ6jB

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–«œ‘«”’ŒŒ•a/ œ‘‘B«Žīį‡‚ĢƒAƒ‹ƒSƒŠƒYƒ€ CQ7 CQ11 CQ9 CQ10 CQ8

2DMPN ‚ĢƒAƒ‹ƒSƒŠƒYƒ€

PVCETCPMF ‚šf’f‚µCƒŠƒXƒN•Ź‚ÉŽ”—Ć•ūj‚š—§‚Ă邱‚Ę‚ŖŠī–{‚Ę‚Č‚éB

PV ‚ĘET ‚ĢŽ”—Ć–Ś•W‚ĶCŒŒšĒ‚āoŒŒ‚š—\–h‚·‚邱‚Ę‚Å‚ ‚éB‘S‚Ä‚ĢƒŠƒXƒNƒJƒeƒSƒŠ[‚É‘®‚·‚éPVŠ³ŽŅ‚É‘Ī‚µ‚Ä’į—p—ŹƒAƒXƒsƒŠƒ““Š—^iCQ8j‚ĘąbŒŒiCQ7j‚Ŗ—LŒų‚Å‚ ‚éBLow ƒŠƒXƒNETiƒ60 ĪC‚©‚ĀŒŒšĒ‚ĢŠł‰‚Ŗ‚Č‚¢j‚Ģ’†‚ŁCSŒŒŠĒƒŠƒXƒNƒtƒ@ƒNƒ^[i‹i‰ŒC‚ŒŒˆ³C‚ƒRƒŒƒXƒeƒ[ƒ‹ŒŒĒC“œ”A•aj‚Ģ‚ ‚éĒ—įCJAK2 •ĻˆŁ‚Ģ‚ ‚éĒ—į‚Å‚ĶCŒŒšĒ”­ĒƒŠƒXƒN‚š’į‰ŗ‚³‚¹‚邽‚ߍRŒŒ¬”Ā—Ć–@iƒAƒXƒsƒŠƒ““Š—^j‚š„§‚·‚éiCQ9jB‚±‚ź‚ē‚ĢƒŠƒXƒN‚Ģ‚Č‚¢Low ƒŠƒXƒNET ‚É‘Ī‚·‚éRŒŒ¬”Ā—Ć–@‚Ģ—L—p«‚Ķ•s–¾‚Å‚ ‚čC‚³‚ē‚ÉŽį”NŽŅ‚É‘Ī‚µ‚Ä‚Ķ‰»Šw—Ć–@‚É‚ę‚é”­‚Ŗ‚ń«‚ąS”z‚³‚ź‚邱‚Ę‚©‚ēCˆź•”‚ĢŠ³ŽŅ‚Å‚Ķ–³Ž”—ĆŒo‰ßŠĻŽ@‚Ģ•ūj‚ą‘I‘š‚³‚ź‚éiCQ10jBˆź•ūCHigh ƒŠƒXƒN‚ĢPV ‚āET Ē—įi60 ĪˆČć‚Ü‚½‚ĶŒŒšĒ‚ĢŠł‰‚ ‚čj‚É‚ĶƒqƒhƒƒLƒVƒEƒŒƒAiHUj‚Ģ“Š—^‚šs‚Į‚Ä‚ą‚ę‚¢iCQ9jB”DP‡•¹ET ‚É‘Ī‚·‚é’į—p—ŹƒAƒXƒsƒŠƒ“‚āƒCƒ“ƒ^[ƒtƒFƒƒ“ƒæiIFNƒæj‚É‚ę‚鎔—Ɖī“ü‚Ķ—¬ŽY‚šŒø‚ē‚·‰Ā”\«‚Ŗ‚ ‚éiCQ10jBPMF ‚É‘Ī‚µ‚Ä‚Ķ–ņ•Ø—Ć–@‚ŖPMF ‚Ģ—\Œć‚š‰ü‘P‚·‚é‚©‚ÉŠÖ‚µ‚Ä‚ĶŒ»Žž“_‚Å•s–¾‚Å‚ ‚é‚Ģ‚ŁC•nŒŒC‘SgŒ‘‘ÓŠ“CäBŽī‚É”ŗ‚¤• •”–c–žŠ“‚Č‚Ē‚Ŗ‚ ‚鏼‡‚ĶCĒóŠÉ˜a‚š–Ś“I‚Ę‚µ‚½Ž”—Ć‚šs‚¤BĒó‰ü‘P‚ĢŽč’i‚Ģˆź‚Ā‚Ę‚µ‚ÄHU ‚Č‚Ē‚š—p‚¢‚½‰»Šw—Ć–@‚Ŗ—L—p‚Å‚ ‚éB‚Ü‚½CJAK2 ‘jŠQÜ‚Č‚Ē‚Ģ—Տ°ŽŽŒ±‚ÉŽQ‰Į‚·‚邱‚Ę‚ą‚Å‚«‚éBĒó‚Ģ‚Č‚¢ź‡‚Ķ–³Ž”—ĆŒo‰ßŠĻŽ@‚Ģ•ūj‚Ŗ–]‚Ü‚µ‚¢iCQ10jBPMF ‚É‘Ī‚·‚éŖŽ”“IŽ”—Ć–@‚Ķallo-HSCT ‚Å‚ ‚éB—\Œć—\‘Ŗƒ‚ƒfƒ‹‚É‚ę‚č—\Œć•s—Ē‚Ę”»’f‚³‚źC“KŲ‚Čƒhƒi[‚Ŗ‘¶Ż‚·‚鏼‡‚ĶCallo-HSCT ‚Ģ“K‰ž‚šl—¶‚·‚é•K—v‚Ŗ‚ ‚éiCQ11jB

y‘ę1.1”ŏC³z

–«œ‘«”’ŒŒ•a/ œ‘‘B«Žīį‡‚ĢƒAƒ‹ƒSƒŠƒYƒ€ CQ7 CQ8 CQ10 CQ9 ’Ē‰ĮCQ CQ11

2DMPN ‚ĢƒAƒ‹ƒSƒŠƒYƒ€

PVCETCPMF ‚šf’f‚µCƒŠƒXƒN•Ź‚ÉŽ”—Ć•ūj‚š—§‚Ă邱‚Ę‚ŖŠī–{‚Ę‚Č‚éB

PV ‚ĘET ‚ĢŽ”—Ć–Ś•W‚ĶCŒŒšĒ‚āoŒŒ‚š—\–h‚·‚邱‚Ę‚Å‚ ‚éB‘S‚Ä‚ĢƒŠƒXƒNƒJƒeƒSƒŠ[‚É‘®‚·‚éPVŠ³ŽŅ‚É‘Ī‚µ‚Ä’į—p—ŹƒAƒXƒsƒŠƒ““Š—^‚ĘąbŒŒiCQ7j‚Ŗ—LŒų‚Å‚ ‚éBLow ƒŠƒXƒNETiƒ60 ĪC‚©‚ĀŒŒšĒ‚ĢŠł‰‚Ŗ‚Č‚¢j‚Ģ’†‚ŁCSŒŒŠĒƒŠƒXƒNƒtƒ@ƒNƒ^[i‹i‰ŒC‚ŒŒˆ³C‚ƒRƒŒƒXƒeƒ[ƒ‹ŒŒĒC“œ”A•aj‚Ģ‚ ‚éĒ—įCJAK2 •ĻˆŁ‚Ģ‚ ‚éĒ—į‚Å‚ĶCŒŒšĒ”­ĒƒŠƒXƒN‚š’į‰ŗ‚³‚¹‚邽‚ߍRŒŒ¬”Ā—Ć–@iƒAƒXƒsƒŠƒ““Š—^j‚š„§‚·‚éiCQ8jB‚±‚ź‚ē‚ĢƒŠƒXƒN‚Ģ‚Č‚¢Low ƒŠƒXƒNET ‚É‘Ī‚·‚éRŒŒ¬”Ā—Ć–@‚Ģ—L—p«‚Ķ•s–¾‚Å‚ ‚čC‚³‚ē‚ÉŽį”NŽŅ‚É‘Ī‚µ‚Ä‚Ķ‰»Šw—Ć–@‚É‚ę‚é”­‚Ŗ‚ń«‚ąS”z‚³‚ź‚邱‚Ę‚©‚ēCˆź•”‚ĢŠ³ŽŅ‚Å‚Ķ–³Ž”—ĆŒo‰ßŠĻŽ@‚Ģ•ūj‚ą‘I‘š‚³‚ź‚éiCQ9jBˆź•ūCHigh ƒŠƒXƒN‚ĢPV ‚āET Ē—įi60 ĪˆČć‚Ü‚½‚ĶŒŒšĒ‚ĢŠł‰‚ ‚čj‚É‚ĶƒqƒhƒƒLƒVƒEƒŒƒAiHUj‚Ģ“Š—^‚šs‚Į‚Ä‚ą—Ē‚¢iCQ9jBHU •s‘Ļ—eA‚ ‚é‚¢‚Ķ’ļR«‚ĢHigh ƒŠƒXƒNET ‚É‚ĶAƒAƒiƒOƒŒƒŠƒhi{ƒAƒXƒsƒŠƒ“j‚š“Š—^‚·‚éB”DP‡•¹ET‚É‘Ī‚·‚é’į—p—ŹƒAƒXƒsƒŠƒ“‚āƒCƒ“ƒ^[ƒtƒFƒƒ“ƒæ(IFNƒæ)‚É‚ę‚鎔—Ɖī“ü‚Ķ—¬ŽY‚šŒø‚ē‚·‰Ā”\«‚Ŗ‚ ‚é (CQ10)BPMF ‚É‘Ī‚µ‚Ä‚Ķ–ņ•Ø—Ć–@‚ŖPMF ‚Ģ—\Œć‚š‰ü‘P‚·‚é‚©‚ÉŠÖ‚µ‚Ä‚ĶŒ»Žž“_‚Å•s–¾‚Å‚ ‚é‚Ģ‚ŁC•nŒŒC‘SgŒ‘‘ÓŠ“CäBŽī‚É”ŗ‚¤• •”–c–žŠ“‚Č‚Ē‚Ŗ‚ ‚鏼‡‚ĶCĒóŠÉ˜a‚š–Ś“I‚Ę‚µ‚½Ž”—Ć‚šs‚¤BĒó‰ü‘P‚ĢŽč’i‚Ģˆź‚Ā‚Ę‚µ‚ÄHU ‚Č‚Ē‚š—p‚¢‚½‰»Šw—Ć–@‚Ŗ—L—p‚Å‚ ‚éBPMF ‚É‘Ī‚·‚éŖŽ”“IŽ”—Ć–@‚Ķallo-HSCT ‚Å‚ ‚éB—\Œć—\‘Ŗƒ‚ƒfƒ‹‚É‚ę‚čHigh ƒŠƒXƒN‚Ę”»’f‚³‚źA“KŲ‚Čƒhƒi[‚Ŗ‘¶Ż‚·‚鏼‡‚ĶAallo-HSCT ‚Ģ“K‰ž‚šl—¶‚·‚éiCQ11jB“KŲ‚Čƒhƒi[‚Ŗ“¾‚ē‚ź‚øA‚©‚Ā‘SgŒ‘‘ÓŠ“AäBŽī‚É”ŗ‚¤• •”Ēó‚Č‚Ē‚Ŗ‚ ‚鏼‡‚ĶAƒ‹ƒLƒ\ƒŠƒ`ƒjƒu‚š“Š—^‚·‚éi’Ē‰ĮCQjBLow ƒŠƒXƒN‚Å‚ ‚čA‚©‚ĀĒó‚Ģ‚Č‚¢ź‡‚Ķ–³Ž”—ĆŒo‰ßŠĻŽ@‚Ģ•ūj‚Ŗ–]‚Ü‚µ‚¢iCQ9jB



CQ1

‰”­CML-CP ‚É‘Ī‚·‚鎔—Ć‚Ę‚µ‚ĉ½‚š“Š—^‚·‚ׂ«‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[1
CML-CP ‚É‘Ī‚µ‚Ä‚ĶCTKI ‚Å‚ ‚éƒCƒ}ƒ`ƒjƒu400 mg QDi1 ‰ń/“śjCƒjƒƒ`ƒjƒu300 mgBIDi2 ‰ń/“śjCƒ_ƒTƒ`ƒjƒu100 mg QD ‚Ģ‚¢‚ø‚ź‚©‚Ģ“Š—^‚š„§‚·‚éB3 Ü‚Ģ•›ģ—pƒvƒƒtƒ@ƒCƒ‹‚ŖˆŁ‚Ȃ邱‚Ę‚©‚ēC‡•¹Ž¾Š³‚Č‚Ē‚ĢŠ³ŽŅ”wŒi‚šl—¶‚µ‚ÄŽ”—Ć–ņ‚š‘I‘š‚·‚邱‚Ę‚Ŗ–]‚Ü‚µ‚¢B

y‰š ąz

‰”­CML-CP ‚É‘Ī‚µ‚Ä‚ĶCTKI ‚Å‚ ‚éƒCƒ}ƒ`ƒjƒu‚ʉ»Šw—Ć–@{ƒCƒ“ƒ^[ƒtƒFƒƒ“ƒæiIFNƒæj‚Ģ•¹—p—Ć–@‚Ę‚Ģ”äŠrŽŽŒ±iIRIS ŽŽŒ±j‚ĢŒ‹‰ŹCƒCƒ}ƒ`ƒjƒu‚Ģ—DˆŹ«‚ŖŽ¦‚³‚ź‚½1jBƒCƒ}ƒ`ƒjƒu“Š—^‚É‚ę‚é8 ”NŠŌ‘S¶‘¶Š„‡iOSj‚Ķ85“iCML ŠÖŒWŽ€‚É‚ę‚鎀–S‚Ģ‚Ż‚š‘ĪŪ‚Ę‚µ‚½8 ”NŠŌOS ‚Ķ93“j‚Ę’·ŠśŠŌ‚Ģ—LŒų«‚ĘˆĄ‘S«‚ąŽ¦‚³‚ź‚½2jB‚»‚ĢŒćC‚—p—Źi800 mg QDjƒCƒ}ƒ`ƒjƒu‚Ę’Źķ—p—Źi400 mg QDjƒCƒ}ƒ`ƒjƒu‚Ģ”äŠrŽŽŒ±‚ŖŽĄŽ{‚³‚ź‚½‚ŖC—¼ŒQ‚Ģ—LŒų«‚ÉŠÖ‚·‚鍷‚Ķ–¾‚ē‚©‚Å‚Č‚¢3j`5jB‚µ‚½‚Ŗ‚Į‚ÄŒ»Žž“_‚Å‚ĶCƒCƒ}ƒ`ƒjƒu400 mg QD ‚Ŗ„§“Š—^‚Ģˆź‚Ā‚Å‚ ‚éB

ƒCƒ}ƒ`ƒjƒu‚š‘ĪĘ–ņ‚Ę‚µ‚Ä‘ę2 ¢‘ćTKI ‚Å‚ ‚éƒjƒƒ`ƒjƒuCƒ_ƒTƒ`ƒjƒu‚Ģ—Տ°‘ęⅢ‘ŠŽŽŒ±‚Ŗ”­•\‚³‚ź‚Ä‚¢‚éBƒjƒƒ`ƒjƒu300 mg BIDiENESTnd ŽŽŒ±j6jCƒ_ƒTƒ`ƒjƒu100 mg QDiDASISION ŽŽŒ±j7j‚ĶC×–Eˆā“`Šw“IŠ®‘S‘tŒųiCCyRjC•ŖŽqˆā“`Šw“I‘å‘tŒųiMMRj’B¬—¦‚É‚Ā‚¢‚Ä12 ƒJŒŽŽž“_‚ŃCƒ}ƒ`ƒjƒu400 mg QD ‚ę‚č—D‚ź‚Ä‚¢‚½B24 ƒJŒŽ‚Ü‚Å‚ĢŒö•\‚³‚ź‚½ƒf[ƒ^‚É‚ę‚é‚ĘAP/BP ‚Ö‚ĢˆŚs‚ą­‚Č‚¢8j‚ŖC’·ŠśŠŌ‚Ģ—LŒų«‚āˆĄ‘S«‚Ģ•]‰æ‚Ŗ•K—v‚Å‚ ‚éB

ÅV‚ĢELN 2013 ƒRƒ“ƒZƒ“ƒTƒX”Å‚Å‚ĶC‘ę2 ¢‘ć‚Ģƒjƒƒ`ƒjƒu‚Ęƒ_ƒTƒ`ƒjƒu“ÆŽm‚š’¼Ś”äŠr‚µ‚½ŒŸ“¢‚ą‚Č‚¢‚½‚߁CŒ»Žž“_‚Å‚Ķ3 Ü‚Ģ‚¤‚æ‚Ē‚ź‚š“Š—^‚·‚ׂ«‚©’f’č‚³‚ź‚Ä‚¢‚Č‚¢9jB‚µ‚©‚µC‘ę2 ¢‘ćTKI ‚Ŗ‘¬‚ā‚©‚Å”ńķ‚ɐ[‚¢•ŖŽqˆā“`Šw“I‘tŒųiMRj‚š‚ą‚½‚ē‚·—˜“_‚šl—¶‚µCŽŠ“K‘tŒų’B¬‚Ģ—L–³‚ĶELN 2009 ƒRƒ“ƒZƒ“ƒTƒX”Å6j‚ę‚葁‚¢Žž“_‚Å”»’f‚³‚ź‚éBTKI 3 Ü‚Ģ•›ģ—pƒvƒƒtƒ@ƒCƒ‹‚ŖˆŁ‚Ȃ邱‚Ę‚©‚ēC‡•¹‚·‚鎾Š³‚Č‚ĒŠ³ŽŅ”wŒi‚šl—¶‚µCŽ”—Ć–ņ‚š‘I‘š‚·‚邱‚Ę‚Ŗ–]‚Ü‚µ‚¢B

yŽQl•¶Œ£z

1j OfBrien SG, et al. Imatinib compared with interferon and low-dose cytarabine for newly diagnosed chronic- phase chronic myeloid leukemia. N Engl J Med. 2003 ; 348i11j : 994-1004.i1iiDivj

2j Deininger M, et al. International randomized study of interferon vs STI571iIRISj 8-year follow up : sustained survival and low risk for progression or events in patients with newly diagnosed chronic myeloid leukemia in chronic phaseiCML-CPj treated with imatinib. Blood. 2009 ; 114 : abstract #1126.i2Diiij

3j Baccarani M, et al. Comparison of imatinib 400 mg and 800 mg daily in the front-line treatment of highrisk, Philadelphia-positive chronic myeloid leukemia : a European LeukemiaNet Study. Blood. 2009 ; 113i19j : 4497-504.i1iiDivj

4j Cortes JE, et al. Phase Ⅲ, Randomized, open-label study of daily imatinib mesylate 400 mg versus 800 mg in patients with newly diagnosed, previously untreated chronic myeloid leukemia in chronic phase using molecular end points : tyrosine kinase inhibitor optimization and selectivity study. J Clin Oncol. 2010 ; 28i3j : 424-30.i1iiDivj

5j Hehlmann R, et al. Tolerability-Adapted Imatinib 800mg/d Versus 400mg/d Versus 400mg/d Plus Interferon- alpha in Newly Diagnosed Chronic Myeloid Leukemia. J Clin Oncol. 2011 ; 29 i12j : 1634-42.i1iiDivj

6j Saglio G, et al. Nilotinib versus imatinib for newly diagnosed chronic myeloid leukemia. N Engl J Med. 2010 ; 362i24j : 2251-9.i1iiDivj

7j Kantarjian H, et al. Dasatinib versus imatinib in newly diagnosed chronic-phase chronic myeloid leukemia. N Eng J Med. 2010 ; 362i24j : 2260-70.i1iiDivj

8j Kantarjian HM, et al. Nilotinib versus imatinib for the treatment of patients with newly diagnosed chronic phase, Philadelphia chromosome-positive, chronic myeloid leukemia : 24-month minimum follow-up of the phase 3 randomized ENESTnd trial. Lancet Oncol. 2011 ; 12i9j : 841-51.i1iiDiiij

9j Beccarani M, et al. European LeukemiaNet recommendations for the management of chronic myeloid leukemia : 2013. Blood. 2013 ; 122i6j : 872-84.iƒŒƒrƒ…[j


CQ2

ƒCƒ}ƒ`ƒjƒu‚É‚ÄOptimal ‚ČŒų‰Ź‚Ŗ“¾‚ē‚ź‚Ä‚¢‚éCML-CP Ē—į‚ĶƒCƒ}ƒ`ƒjƒu‚šŒp‘±‚·‚ׂ«‚©C‘ę2 ¢‘ćTKI ‚É•ĻX‚·‚é‚Ł‚¤‚Ŗ‚ę‚¢‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2A
ƒCƒ}ƒ`ƒjƒu‚ĢŒp‘±Ž”—Ć‚š„§‚·‚éBƒCƒ}ƒ`ƒjƒu‚Ģ•›ģ—p‚Č‚Ē‚É‚ę‚čCƒAƒhƒqƒAƒ‰ƒ“ƒXi“ą•žŽ”—Ć‚ĢŒp‘±«j‚Ģ’į‰ŗ‚ŖŠėœœ‚³‚ź‚鏼‡‚ĶC•›ģ—p‚Ģƒvƒƒtƒ@ƒCƒ‹‚ĢˆŁ‚Č‚é‘ę2 ¢‘ćTKI ‚É•ĻX‚·‚邱‚Ę‚ą‰Ā”\‚Å‚ ‚éB

y‰š ąz

2009 ”NELN ƒRƒ“ƒZƒ“ƒTƒX‰ü’ł”Å‚É‚ę‚égOptimal responseh‚ĶCƒCƒ}ƒ`ƒjƒu“Š—^12 ƒJŒŽŽž“_‚Å‚Ģ×–Eˆā“`Šw“IŠ®‘S‘tŒųiCCyRj’B¬‚Ø‚ę‚Ń18 ƒJŒŽŽž“_‚Å‚Ģ•ŖŽqˆā“`Šw“I‘å‘tŒųiMMRj’B¬‚Ę‹K’č‚³‚ź‚Ä‚¢‚é1jBIRIS ŽŽŒ±‚ĢƒTƒuƒZƒbƒg‰šĶ‚Å‚ĶCƒCƒ}ƒ`ƒjƒu“Š—^Œć12 ƒJŒŽCCyR ’B¬—į‚Ģ5 ”N–³‘ˆ«¶‘¶Š„‡iPFSj‚Ķ97“‚Å‚ ‚čC18 ƒJŒŽ‚ÅMMR ’B¬—į‚Ģ7 ”N–³ƒCƒxƒ“ƒg¶‘¶Š„‡iEFSj95“ , PFS 99“‚Ę‹É‚ß‚Ä—ĒD‚Å‚ ‚éBƒCƒ}ƒ`ƒjƒu“Š—^Œć12 ƒJŒŽ‚ÅMMR ’B¬—į‚ĶCMLAP/BP ‚Ö‚ĢˆŚs‚Ŗ8 ”NŽž“_‚Ü‚Å•ń‚³‚ź‚Ä‚¢‚Č‚¢2j`4jB‚±‚Ģ‚ꂤ‚ÉOptimal Ē—į‚ĶCƒCƒ}ƒ`ƒjƒuŒp‘±‚É‚ę‚č—ĒD‚Č’·Šś—\Œć‚Ŗ“¾‚ē‚ź‚é‚ʍl‚¦‚ē‚ź‚éB

ƒCƒ}ƒ`ƒjƒu“Š—^‚É‚ę‚éOptimal Ē—į‚š‘ę2 ¢‘ćTKI ‚É•ĻX‚·‚é‚©‚É‚Ā‚¢‚Ä‚ĶCƒCƒ}ƒ`ƒjƒuŒp‘±“Š—^ŒQ‚Ę‘ę2 ¢‘ćTKI ‚Ö‚Ģ•ĻXŒQ‚Ę‚Ģ—Տ°”äŠrŽŽŒ±‚š‚·‚邱‚Ę‚ÅC–¾‚ē‚©‚É‚Č‚é‚ʍl‚¦‚ē‚ź‚éBŒ»ŻCƒCƒ}ƒ`ƒjƒu‚š2 ”NˆČć“Š—^‚µ•ŖŽqˆā“`Šw“IŠ®‘S‘tŒųiCMRj‚É“ž’B‚µ‚Ä‚¢‚Č‚¢Ē—į‚šCƒjƒƒ`ƒjƒu400 mg BID ŒQ‚ĘƒCƒ}ƒ`ƒjƒuˆŪŽŒQ‚Ƀ‰ƒ“ƒ_ƒ€‰»‚µCCMR ‚šƒGƒ“ƒhƒ|ƒCƒ“ƒg‚Ę‚µ‚½—Տ°ŽŽŒ±iENESTcmrj‚Ŗis’†‚Å‚ ‚čC24 ƒJŒŽŽž“_‚Å‚ĢŒ‹‰Ź‚Ŗ”­•\‚³‚ź‚½5jBƒjƒƒ`ƒjƒu‚Ö•ĻX‚·‚邱‚Ę‚Å12 ƒJŒŽŽž“_‚ĢCMR ’B¬—¦‚Ŗ—LˆÓ‚É‘‰Į‚µi23“ vs 11“Cp0.02jC24 ƒJŒŽŽž“_‚Å‚Ķ‚³‚ē‚É‘‰Įi32.7“ vs 16.5“Cp0.005j‚µCBCR-ABL1 ‚ŖŒŸoŒĄŠEˆČ‰ŗ‚Ģ’B¬—¦‚Ŗ—LˆÓ‚ɍ‚‚­‚Č‚Į‚½i22.1“ vs 8.7“Cp0.0087jB‚µ‚©‚µC‚±‚ĢŒ‹‰Ź‚Ŗ–ņÜ’†Ž~‚É‚Ā‚Č‚Ŗ‚é‚Ģ‚©‚Ķ”Œć‚Ģ’·ŠśŠĻŽ@‚š—v‚·‚éB

‚Č‚ØCMMR ‚ĢˆŪŽ‚ĘƒAƒhƒqƒAƒ‰ƒ“ƒX‚É‚Ķ‹­‚¢‘ŠŠÖ‚Ŗ‚ ‚čC90“ˆČćƒCƒ}ƒ`ƒjƒu‚š•ž–ņ‚Å‚«‚½ź‡‚Ķ—LˆÓ‚ÉOptimal response ‚šˆŪŽ‚Å‚«‚é‚Ę‚¢‚¤6jB‚µ‚©‚µ‚Č‚Ŗ‚ēCOptimal Ē—į‚É‚Ø‚¢‚Ä‚ą•›ģ—p‚É‘Ļ‚¦‚Č‚Ŗ‚ēƒCƒ}ƒ`ƒjƒu‚šŒp‘±‚µ‚Ä‚¢‚é•s‘Ļ—eĒ—į‚Ŗ‘¶Ż‚·‚邽‚߁C‚±‚Ģ‚ꂤ‚ȏĒ—į‚É‚Ķ•›ģ—p‚Ģƒvƒƒtƒ@ƒCƒ‹‚ŖˆŁ‚Č‚é‘ę2 ¢‘ćTKI ‚ÖŽ”—Ć•ĻX‚·‚é‚Ģ‚ąˆź‚Ā‚Ģ‘I‘šŽˆ‚ʍl‚¦‚ē‚ź‚éB

ELN 2013 ƒRƒ“ƒZƒ“ƒTƒX”Å‚Å‚ĶC•›ģ—p‚Č‚Ē‚ŃCƒ}ƒ`ƒjƒu‚ĢŽŠ“K‘tŒų‚Ŗ“¾‚ē‚ź‚Č‚¢•s‘Ļ—e—į‚Č‚Ē‚Å‚ĶC‘Šś‚Ƀjƒƒ`ƒjƒu‚āƒ_ƒTƒ`ƒjƒu‚Ö‚Ģ•ĻX‚š„§‚µ‚Ä‚¢‚é7jB

yŽQl•¶Œ£z

1j Baccarani M, et al. Chronic myeloid leukemia : an update of concepts and management recommendations of European LeukemiaNet. J Clin Oncol. 2009 ; 27i35j : 6041-51.iƒŒƒrƒ…[j

2j Druker BJ, et al. Five-year follow-up of patients receiving imatinib for chronic myeloid leukemia. N Engl J Med. 2006 ; 355i23j : 2408-17.i2Diiij

3j Huges TP, et al. Long-term prognostic significance of early molecular response to imatinib in newly diagnosed chronic myeloid leukemia : an analysis from the International Randomized Study of Interferon and STI571iIRISj. Blood. 2010 ; 116i19j : 3758-65.i2Diiij

4j Deininger M, et al. International randomized study of interferon vs STI571iIRISj 8-year follow-up : sustained survival and low risk for progression or events in patients with newly diagnosed chronic myeloid leukemia in chronic phaseiCML-CPj treated with imatinib. Blood. 2009 ; 114 : abstract #1126.i2Diiij

5j Hughes TP, et al. Switching to Nilotinib Is Associated with Continued Deeper Molecular Responses in CML-CP Patients with Minimal Residual Disease After 2 Years On Imatinib : Enestcmr 2-Year Followup Results BloodiASH Annual Meeting Abstractsj. 2012 ; 120 : abstract 694.i1iiDivj

6j Marin D, et al. Adherence is the critical factor for achieving molecular responses in patients with chronic myeloid leukemia who achieve complete cytogenetic responses on imatinib. J Clin Oncol. 2010 ; 28i14j : 2381-8.i3iiDivj

7j Baccarani M, et al. European LeukemiaNet recommendations for the management of chronic myeloid leukemia : 2013. Blood. 2013 ; 122i6j : 872-84.iƒŒƒrƒ…[j


CQ3

Warning ‚āFailure Ē—į‚É‘Ī‚µ‚ăCƒ}ƒ`ƒjƒu‚Ģ‘—Ź‚Ę‘ę2 ¢‘ćTKI ‚Ģ‚Ē‚æ‚ē‚š‘I‘š‚·‚ׂ«‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2A
ƒCƒ}ƒ`ƒjƒu“Š—^‚ÅFailure —į‚ĶƒCƒ}ƒ`ƒjƒu‘—Ź‚ę‚č‚ą‘ę2 ¢‘ćTKI‚Ö‚Ģ•ĻX‚š„§‚·‚éB

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ƒJƒeƒSƒŠ[2A
ƒCƒ}ƒ`ƒjƒu“Š—^‚ÅWarning —į‚É‚ĶCƒ‚ƒjƒ^ƒŠƒ“ƒO‚š•p‰ń‚ɍs‚¢Failure ‚Ę‚Č‚ź‚Ī‘ę2 ¢‘ćTKI ‚Ö‚Ģ•ĻX‚š„§‚·‚éB

y‰š ąz

ƒCƒ}ƒ`ƒjƒuFailure ‚É‘Ī‚µ‚ăCƒ}ƒ`ƒjƒu400 mg ‚š800 mg ‚ɁC300 mg ‚š600 mg ‚É‘—Ź‚µ‚½ź‡C61 ƒJŒŽ‚ĢŠĻŽ@’†‰›ŠśŠŌ‚É‚Ø‚¢‚čזEˆā“`Šw“IŠ®‘S‘tŒųiCCyRj’B¬—¦40“C3 ”N–³ƒCƒxƒ“ƒg¶‘¶Š„‡iEFSjC‘S¶‘¶Š„‡iOSj‚Ķ47“C76“‚Ę—ĒD‚Å‚ ‚Į‚½‚Ę‚Ģ•ń‚Ŗ‚Č‚³‚ź‚Ä‚¢‚é1jB‘ę2 ¢‘ćTKI ‚ÉŠÖ‚µ‚Ä‚ĶCƒjƒƒ`ƒjƒu400 mg BID ‚É•ĻX‚µ‚½ź‡C24 ƒJŒŽŒć‚ĢCCyR ’B¬—¦‚Ķ44“‚Å‚ ‚čC‚»‚Ģ‚¤‚æ‚Ģ56“‚Ŗ•ŖŽqˆā“`Šw“I‘å‘tŒųiMMRj‚š’B¬‚µOS ‚Ķ87“‚Ę—ĒD‚Å‚ ‚Į‚½2jBƒ_ƒTƒ`ƒjƒu‚ÉŠÖ‚µ‚Ä‚ĶCƒCƒ}ƒ`ƒjƒuFailure ‚É‘Ī‚µ‚āCƒ_ƒTƒ`ƒjƒu70 mg BID ŒQ‚ĘƒCƒ}ƒ`ƒjƒu‘å—Źi400 mg BIDjŒQ‚Ƀ‰ƒ“ƒ_ƒ}ƒCƒY‚µ‚½‘ęⅡ‘ŠŽŽŒ±iSTART-Rj‚Å‚ĶC2 ”NŠŌ‚ĢŠĻŽ@ŠśŠŌ‚É‚Ø‚¢‚ÄCCyR ’B¬‚Ķƒ_ƒTƒ`ƒjƒuŒQ44“CƒCƒ}ƒ`ƒjƒu‘å—ŹŒQ18“‚Å‚ ‚Į‚½ip0.0025jB18 ƒJŒŽŽž“_‚Å‚ĢMMR ’B¬‚ĶŠeX29“C12“ip0.028j‚Å‚ ‚Į‚½3jBˆČć‚ę‚čCƒCƒ}ƒ`ƒjƒuFailure ‚É‘Ī‚µ‚Ä‚ĶƒCƒ}ƒ`ƒjƒu‘—Ź‚ę‚č‚ą‘ę2 ¢‘ćTKI ‚Ģ”½‰ž‚Ŗ—ĒD‚Å‚ ‚Į‚½BŠĻŽ@ŠśŠŌ‚Ŗ’Z‚¢‚ą‚Ģ‚ĢC–³‘ˆ«¶‘¶Š„‡iPFSj‚Ģ‰„’·‚ąŽ¦“‚³‚ź‚邱‚Ę‚©‚ēCƒCƒ}ƒ`ƒjƒuFailure ‚É‘Ī‚µ‚Ä‚Ķ‘ę2 ¢‘ćTKI ‚Ŗ„§‚³‚ź‚éB

y‘ę1.1 ”Å’Ē‰ĮƒRƒƒ“ƒgz

‚Ü‚½AV‹K‘ę“ń¢‘ćTKI ‚Ģƒ{ƒXƒ`ƒjƒu‚Ķę‚É“Š—^‚µ‚½TKIiƒCƒ}ƒ`ƒjƒuAƒjƒƒ`ƒjƒuA‚Ü‚½‚Ķƒ_ƒTƒ`ƒjƒujŽ”—Ć‚É’ļR«E•s‘Ļ—e‚ĢCML ‚É‘Ī‚·‚鎔—Ć–ņ‚Å‚ ‚éBƒCƒ}ƒ`ƒjƒu600mg QD ‚ĢFailure Ē—įin=200j‚šƒ{ƒXƒ`ƒjƒu500mg QD ‚É•ĻX‚µ‚½ź‡A24 ƒ–ŒŽ‚ĢŠĻŽ@ŠśŠŌ‚Ģ—ŻĻCCyR ’B¬—¦‚Ķ46%A—ŻĻMMR’B¬—¦‚Ķ34%‚Å‚ ‚čA2 ”NOS ‚Ķ88%‚Ę—ĒD‚Å‚ ‚Į‚½9jBƒCƒ}ƒ`ƒjƒuFailure ‚É‘Ī‚µ‚ă{ƒXƒ`ƒjƒu‚ą‘I‘šŽˆ‚Ģˆź‚Ā‚Ę‚Č‚Į‚½B‚³‚ē‚ɁA‘¼‚ĢTKI ’ļR«CML ‚É‘Ī‚µ‚Ä3rd line ‚Ę‚µ‚Ä‚ą“Š—^‰Ā”\‚Å‚ ‚é10jB

IRIS ŽŽŒ±‚ɂ؂Ƃ郉ƒ“ƒhƒ}[ƒN‰šĶ‚Å‚ĶCƒCƒ}ƒ`ƒjƒu“Š—^6 ƒJŒŽŽž“_‚ōזEˆā“`Šw“I”ń‘tŒųiNo CyRjC×–Eˆā“`Šw“I”÷¬‘tŒųiMini CyRj/ ×–Eˆā“`Šw“I¬‘tŒųiMinor CyRjC×–Eˆā“`Šw“I•”•Ŗ‘tŒųiPCyRjCCCyR ‚É‚Ø‚Æ‚é6 ”NEFS ‚Ķ59, 58, 85, 91“‚Å‚ ‚čCELN 2009 ƒRƒ“ƒZƒ“ƒTƒX8j‚Å’č‹`‚³‚ź‚½Suboptimal response ‚Å‚ ‚éPCyR ‚Ŗ“¾‚ē‚ź‚Ä‚¢‚Č‚¢ź‡‚Ģ—\Œć‚Ķ•s—Ē‚Å‚ ‚é4jB‚µ‚©‚µC18 ƒJŒŽŽž“_‚Å‚ĢMMR –¢’B¬‚ĢSuboptimal response ‚ĶOptimal response ‚Ę’·Šś—\Œć‚Ķ“Æ“™‚Å‚ ‚é5jB‰p‘‚ĢHammersmith •a‰@‚Ģƒf[ƒ^‚Å‚ĶC3, 6, 12 ƒJŒŽ‚ĢSuboptimal response ‚Å‚ĢPFS ‚ĶFailure ‚É‹ß‚­C18 ƒJŒŽŽž“_‚Å‚ĢSuboptimal response ‚ĘOptimal response ‚Ķ“Æ“™‚ĢPFS ‚Å‚ ‚Į‚½6jBMD ƒAƒ“ƒ_[ƒ\ƒ“‚Ŗ‚ńƒZƒ“ƒ^[‚ĢŒŸ“¢‚Å‚ąC6, 12 ƒJŒŽ‚ĢSuboptimal response Ē—į‚ĢEFS ‚Ķ•s—Ē‚Å‚ ‚Į‚½‚ŖC18 ƒJŒŽ‚ĢSuboptimal response Ē—į‚ĢEFS ‚ĶOptimal response —į‚Ę“Æ“™‚É—ĒD‚Å‚ ‚Į‚½7jB

CQ2 ‚ĢOptimal Ē—į‚Å‚ąŒ¾‹y‚µ‚½‚ꂤ‚ɁC•›ģ—p‚É‘Ļ‚¦‚Č‚Ŗ‚ēƒCƒ}ƒ`ƒjƒu‚šŒp‘±‚µ‚Ä‚¢‚é•s‘Ļ—eĒ—į‚Ŗ‘¶Ż‚·‚邽‚߁C‚±‚Ģ‚ꂤ‚ȏĒ—į‚É‚Ķ•›ģ—p‚Ģƒvƒƒtƒ@ƒCƒ‹‚ŖˆŁ‚Č‚é‘ę2 ¢‘ćTKI ‚ÖŽ”—Ć•ĻX‚·‚é‚Ģ‚ąˆź‚Ā‚Ģ‘I‘šŽˆ‚ʍl‚¦‚ē‚ź‚éB

yŽQl•¶Œ£z

1j Jabbour E, et al. Imatinib mesylate dose escalation is associated with durable responses in patients with chronic myeloid leukemia after cytogenetic failure on standard-dose imatinib therapy. Blood. 2009 ; 113i10j : 2154-60.i3iiiDivj

2j Kantarjian HM, et al. Nilotinib is effective in patients with chronic myeloid leukemia in chronic phase after imatinib resistance or intolerance : 24-month follow-up results. Blood. 2011 ; 117 i4j : 1141-5.i3iiiDivj

3j Kantarjian H, et al. Dasatinib or high-dose imatinib for chronic-phase chronic myeloid leukemia resistant to imatinib at a dose of 400 to 600 miligrams daily. Cancer. 2009 ; 115i18j : 4136-47.i3iiiDivj

4j Hochhaus A, et al. Six-year follow-up of patients receiving imatinib for the first-line treatment of chronic myeloid leukemia. Leukemia. 2009 ; 23i6j : 1054-61.i2Diiij

5j Huges TP, et al. Long-term prognostic significance of early molecular response to imatinib in newly diagnosed chronic myeloid leukemia : an analysis from the International Randomized Study of Interferon and STI571iIRISj. Blood. 2010 ; 116i19j : 3758-65.i2Diiij

6j Marin D, et al. European LeukemiaNet criteria for failure or suboptimal response reliably identify patients with CML in early chronic phase treated with imatinib whose eventual outcome is poor. Blood. 2008 ; 112i12j : 4437-44.i3iiDiiij

7j Alvarado Y, et al. Significance of suboptimal response to imatinib, as defined by the European LeukemiaNet,@in the long-term outcome of patients with early chronic myeloid leukemia in chronic phase. Cancer.@2009 ; 115i16j : 3709-18.i3iiDiiij

8j Beccarani M, et al. Chronic myeloid leukemia : an update of concepts and management recommendations@of European LeukemiaNet. J Clin Oncol 2009 ; 27i35j : 6041-51.iƒŒƒrƒ…[j

y‘ę1.1 ”Å’Ē‹Lz

9j Gambacorti-Passerini C, et al. Bosutinib efficacy and safety in chronic phase chronic myeloid leukemia after imatinib resistance or intolerance:minimum 24-month follow-up. Am J Hematol 2014: 89(7): 732-42.i2Diiij

10j Khoury HJ, et al. Bosutinib is active in chronic phase chronic myeloid leukemia after imatinib and dasatinib and/or nilotinib therapy failure.Blood 2014: 119 (15): 3403-12.i2Diiij


CQ4

ƒCƒ}ƒ`ƒjƒuƒgƒ‰ƒt”Z“x‚Ģ–Ś•W’l‚š1,000 ng/mL ‚Ę‚µ‚ăCƒ}ƒ`ƒjƒu—Ć–@‚ĶŠ©‚ß‚ē‚ź‚é‚©


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ƒJƒeƒSƒŠ[2B
CML ‚ĢƒCƒ}ƒ`ƒjƒu—Ć–@‚É‚Ø‚¢‚ÄŽ”—ĆŒų‰Ź‚Ŗ”F‚ß‚ē‚ź‚Č‚¢ź‡CƒCƒ}ƒ`ƒjƒu‚Ģƒgƒ‰ƒt’l‚šŽQl‚É‚·‚邱‚Ę‚Ŗ‚Å‚«‚éB

y‰š ąz

CML –«Šś‚É‘Ī‚·‚éƒCƒ}ƒ`ƒjƒu‚Ģ’ļR«‚Ģ——R‚Ę‚µ‚āCABL1 ˆā“`Žq“_“Ė‘R•ĻˆŁ‚ā•t‰Į“IõF‘ĢˆŁķ‚Č‚Ē”’ŒŒ•a×–E‚Ģ–ā‘č‚Ģ‚Ł‚©ƒCƒ}ƒ`ƒjƒu‚Ģ–ņ•Ø“®‘Ō‚ÉŠÖ‚ķ‚é–ā‘č‚Ŗ’m‚ē‚ź‚Ä‚¢‚éB–ņ—Šw“I‚É”¼ŒøŠś‚Ģ”äŠr“I’·‚¢ƒCƒ}ƒ`ƒjƒu‚Ģ–ņ•Ø“®‘Ō‚Ķƒgƒ‰ƒt’l‚Å•]‰æ‚·‚邱‚Ę‚Ŗ‰Ā”\‚Å‚ ‚čCŽ”—ĆŒų‰Ź‚Ęƒgƒ‰ƒt’l‚Ģ‘ŠŠÖ‚É‚Ā‚¢‚Ä‚¢‚­‚Ā‚©‚ĢƒRƒz[ƒgŒ¤‹†‚Ŗ•ń‚³‚ź‚Ä‚¢‚éB

Picard ‚ē‚Ķ­‚Č‚­‚Ę‚ą12 ƒJŒŽƒCƒ}ƒ`ƒjƒu—Ć–@‚šŽó‚Æ‚Ä‚¢‚銳ŽŅin68j‚ĢƒCƒ}ƒ`ƒjƒuƒgƒ‰ƒt’l‚Ę×–Eˆā“`Šw“IŒų‰Ź‚Ü‚½‚Ķ•ŖŽqˆā“`Šw“IŒų‰Ź‚Ę‚Ģ‘ŠŠÖ‚š‰‚ß‚ÄŽ¦‚µ‚½B‚³‚ē‚ÉROC ‹Čüireceiver operating characteristic curvej‚ę‚čƒCƒ}ƒ`ƒjƒuƒgƒ‰ƒt’l‚Ģ臒l‚š1,002 ng/mL ‚ɐŻ’肵‚½ź‡C•ŖŽqˆā“`Šw“I‘å‘tŒųiMMRj’B¬‚šŠ““x77“C“ĮˆŁ“x71“‚Å—\‘Ŗ‰Ā”\‚Å‚ ‚é‚Ę‚µ‚½1jB‘½”—į‚Ģ‘O•ūŽ‹“IƒRƒz[ƒgŒ¤‹†in351j‚Å‚ ‚éIRIS ŽŽŒ±‚ĢƒTƒu‰šĶ‚ĶŽ”—Ć29 “ś–Ś‚ĢƒCƒ}ƒ`ƒjƒuƒgƒ‰ƒt’l‚Ŗ×–Eˆā“`Šw“IŠ®‘S‘tŒųiCCyRj’B¬‚ÉŠÖŒW‚·‚邱‚Ę‚šŽ¦‚µ‚½2jB“ś–{l‘½”—į‚ĢƒRƒz[ƒgŒ¤‹†in254j‚Å‚ĶƒCƒ}ƒ`ƒjƒuƒgƒ‰ƒt’l‚ĘMMR ’B¬‚Ģ‘ŠŠÖ‚ŖŽ¦‚³‚ź‚½3jB•W€“Š—^—Ź‚Ģ400 mg “ą•žĒ—į‚š‘ĪŪ‚Ę‚µ‚½•””‚ĢƒRƒz[ƒgŒ¤‹†‚É‚Ø‚¢‚āCƒCƒ}ƒ`ƒjƒuƒgƒ‰ƒt’l‚Ŗ1,000 ng/mL@ˆČć‚ĢĒ—į‚Å—LˆÓ‚ÉMMR ’B¬—¦‚Ģ‚‚¢‚±‚Ę‚Ŗ’ĒŽŽ‚³‚ź‚Ä‚¢‚é3j`5jB

‚—p—ŹƒCƒ}ƒ`ƒjƒu‚Ģ”E—e«‚Ģ–ā‘č‚Ķ‚ ‚é‚ą‚Ģ‚ĢCTOPS ŽŽŒ±‚É‚ę‚č•W€—Ź‚Ę‚Ģ”äŠr‚ɂč‚—p—ŹƒCƒ}ƒ`ƒjƒu‚ĢŽ”—ĆŒų‰Ź‚Ģ‘Šś’B¬‚Ŗ•ń‚³‚ź‚Ä‚¢‚é‚Ŗ6jCƒgƒ‰ƒt’l‚šƒ^[ƒQƒbƒg‚Ę‚µ‚ÄŽ”—Ɖī“ü‚šs‚¤ƒ‰ƒ“ƒ_ƒ€‰»”äŠrŽŽŒ±‚Ķ•ń‚³‚ź‚Ä‚¢‚Č‚¢B‚µ‚©‚µ‚Č‚Ŗ‚ēC”äŠr“I‘½”—į‚Ģ•””‚ĢƒRƒz[ƒgŒ¤‹†‚É‚ę‚čƒgƒ‰ƒt’l‚ĘŽ”—ĆŒų‰Ź‚ĢŠÖ˜A«‚ŖŽ¦‚³‚ź‚Ä‚¢‚邱‚Ę‚©‚ēCŠś‘Ņ‚³‚ź‚鎔—ĆŒų‰Ź‚Ŗ“¾‚ē‚ź‚Č‚¢ź‡‚Ķ“_“Ė‘R•ĻˆŁ‰šĶi•ŪŒÆ“K—pŠOj‚ɉĮ‚¦CƒCƒ}ƒ`ƒjƒuŒŒ’†”Z“x‘Ŗ’či•ŪŒÆ“K—pC“Į’č–ņÜŠĒ——æj‚ŖŽ”—Ć“ą—e‚ĢÄl‚Ģ‚Ż‚Č‚ē‚øC•¹—p–ņ‚Ę‚Ģ‘ŠŒŻģ—p‚āƒRƒ“ƒvƒ‰ƒCƒAƒ“ƒX‚ĢŠm”F‚É—L—p‚Å‚ ‚éB

yŽQl•¶Œ£z

1j Picard S, et al. Trough imatinib plasma levels are associated with both cytogenetic and molecular responses to standard-dose imatinib in chronic myeloid leukemia. Blood. 2007 ; 109i8j : 3496-9.i3iiiDivj

2j Larson RA, et al. Imatinib pharmacokinetics and its correlation with response and safety in chronic-phase chronic myeloid leukemia : a sub analysis of the iris study. Blood. 2008 ; 111i8j : 4022-8.i2Divj

3j Takahashi N, et al. Correlation between imatinib pharmacokinetics and clinical response in Japanese patients with chronic-phase chronic myeloid leukemia. Clin Pharmacol Ther. 2010 ; 88i6j : 809-13.i3iDivj

4j Marin D, et al. Adherence is the critical factor for achieving molecular responses in patients with chronic myeloid leukemia who achieve complete cytogenetic responses on imatinib. J Clin Oncol. 2010 ; 28i14j : 2381-8.i3iiDivj

5j Ishikawa Y, et al. Trough plasma concentration of imatinib reflects BCR-ABL kinase inhibitory activity and clinical response in chronic-phase chronic myeloid leukemia : a report from the BINGO study. Cancer Sci. 2010 ; 101i10j : 2186-92.i3iiDivj

6j Cortes JE, et al. Phase Ⅲ, Randomized, open-label study of daily imatinib mesylate 400 mg versus 800 mg in patients with newly diagnosed, previously untreated chronic myeloid leukemia in chronic phase using molecular end points : tyrosine kinase inhibitor optimization and selectivity study. J Clin Oncol. 2010 ; 28i3j : 424-30.i1iiDivj


CQ5

isŠśCMLiAP ‚Ø‚ę‚ŃBPj‚ĢŽ”—Ć‚ĶTKI ‚ŖŠ©‚ß‚ē‚ź‚é‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2A
AP ‚ĢŽ”—Ć‚ĶCƒ`ƒƒVƒ“ƒLƒi[ƒ[‘jŠQÜi‚—p—ŹƒCƒ}ƒ`ƒjƒu‚Ü‚½‚Ķ‘ę2 ¢‘ćTKIj‚š„§‚·‚éBTKI ‚ÅŽŠ“K‘tŒų‚Ŗ“¾‚ē‚ź‚Č‚¢ź‡‚Ķ“ÆŽķHSCT ‚šl—¶‚·‚éB

„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2A
BP ‚ĢŽ”—Ć‚ĶTKI ’PÜ‚Ü‚½‚ĶTKI ‚šŠÜ‚Ž‰»Šw—Ć–@‚ōőåŒų‰Ź‚š“¾‚½ŒćC‰Ā”\‚ČŒĄ‚č“ÆŽķHSCT ‚š„§‚·‚éB

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CML-AP ‚ĢŽ”—Ć‚ĶCTKI ‚ÅŠJŽn‚³‚źCƒCƒ}ƒ`ƒjƒu‚Å‚ ‚ź‚Ī‚—p—Ź600 mgQD ‚Ŗ„§‚³‚ź‚é1j2jBƒCƒ}ƒ`ƒjƒuŽ”—Ć‚ÅŒŒ‰tŠw“I‘tŒųiHRj 82“CŠ®‘SHRiCHRj 34“C×–Eˆā“`Šw“I‘å‘tŒųiMCyRj24“C×–Eˆā“`Šw“IŠ®‘S‘tŒųiCCyRj 17“‚Å‚ ‚čC12 ƒJŒŽŽž“_‚Å‚Ģ–³•a¶‘¶Š„‡iPFSj59“C‘S¶‘¶Š„‡iOSj74“‚Å‚ ‚Į‚½2jBhistorical control ‚Å‚ ‚éƒCƒ“ƒ^[ƒtƒFƒƒ“ƒæiIFNƒæj‚ā‰»Šw—Ć–@‚Ę”äŠr‚µCƒCƒ}ƒ`ƒjƒuŽ”—ĆŒQ‚Ģ4 ”NOS ‚Ķ53“CIFNƒæŒQ42“C‘¼‚ĢCT ŒQ0`21“‚ĘƒCƒ}ƒ`ƒjƒu‚ĢŽ”—Ć—DˆŹ«‚ŖŽ¦‚³‚ź‚Ä‚¢‚é3jBˆź•ūCƒCƒ}ƒ`ƒjƒuŽ”—Ć’ļR«‚ą‚µ‚­‚Ķ•s‘Ļ—eCML-AP Ē—į‚Å‚Ķ‘ę2 ¢‘ćTKI ‚Ŗ—L—p‚Å‚ ‚邽‚߁CŽ”—Ć–ņ‚š‘ę2 ¢‘ćTKI ‚É•ĻX‚·‚éBƒjƒƒ`ƒjƒu‚ɐŲ‚č‘Ö‚¦‚É‚ÄCHR 26“CCCyR 16“iŠĻŽ@’†‰›ŠśŠŌ202 “śj4jCƒ_ƒTƒ`ƒjƒu‚ɐŲ‚č‘Ö‚¦‚É‚ÄCHR 45“CCyR 32“iŠĻŽ@’†‰›ŠśŠŌ14.1 ƒJŒŽj‚Å‚ ‚Į‚½5jBƒjƒƒ`ƒjƒu‚Ģ24 ƒJŒŽŽž“_‚Å‚ĢOS ‚ĘPFS ‚Ķ‚»‚ź‚¼‚ź70“‚Ę33“‚Å‚ ‚Į‚½6jB‚Č‚ØCˆŚA“K‰ž‚Ŗ‚ ‚鏼‡‚ĶTKI ‚É‚ę‚鎔—Ć”½‰ž«‚šŒ©‹É‚ß‚½ć‚ŁCallo-HSCT ‚šl—¶‚µ‚Ä‚ą‚ę‚¢B

CML-BP ‚É‘Ī‚µ‚Ä‚ĶTKI ’PÜ7j8j‚Ü‚½‚ĶAML/ALL ‚ɏ€‚¶‚½‰»Šw—Ć–@‚Ģ•¹—p9j‚ÅŽ”—Ć‚·‚éB‚µ‚©‚µ‚Č‚Ŗ‚ēTKI ’PÜ‚ą‚µ‚­‚Ķ‰»Šw—Ć–@‚ĢŽ”—Ɛ¬Ń‚Ķ\•Ŗ‚Ę‚ĶŒ¾‚¦‚Č‚¢‚½‚߁CˆŚA“K‰ž‚ĢŠ³ŽŅ‚Å‚Ķallo-HSCT ‚Ŗ‹­‚­„§‚³‚ź‚éBƒhƒCƒc‚ĢCML ƒOƒ‹[ƒv‚Ģ•ń‚Å‚ĶisŠś‚ĢCML ‚É‘Ī‚·‚éallo-HSCT ‚Ģ3 ”NOS ‚Ķ59“‚Å‚ ‚é10jB‚Ü‚½CCML ‚É‚Ø‚Æ‚éTKI Ž”—Ć’†‚ÉT315I •ĻˆŁ‚ŖŠm”F‚³‚ź‚½ź‡CŒ»Ż‚ĢTKI ‚Å‚Ķ—Տ°Œų‰Ź‚Ŗ‚Č‚¢‚½‚߁CˆŚA“K‰ž‚Ģ‚ ‚銳ŽŅ‚Å‚Ķallo-HSCT ‚ ‚é‚¢‚Ķ—Տ°ŽŽŒ±‚Ö‚ĢŽQ‰Į‚Ŗ„§‚³‚ź‚éB

ELN 2013 ƒRƒ“ƒZƒ“ƒTƒX11j‚Å‚ĶCAP/BP Šś‚ĢŽ”—Ć•ūj‚Ę‚µ‚āC‘OŽ”—Ć‚ÉTKI “Š—^‚Ģ‚Č‚¢‰”­AP/BP Šś‚Å‚ĶC‚—p—Ź‚ĢƒCƒ}ƒ`ƒjƒui400 mgBIDj‚ą‚µ‚­‚Ķƒ_ƒTƒ`ƒjƒui70 mgBID ‚Ü‚½‚Ķ140 mgQDj‚š‘I‘š‚µCallo-HSCT ‚Ģƒhƒi[’T‚µ‚š„§‚µ‚Ä‚¢‚éB‚»‚µ‚āCBP Šś‚Ķ‰Ā”\‚ČŒĄ‚č‘SˆõCAP Šś‚ĶOptimal ‘tŒų‚Ŗ“¾‚ē‚ź‚Č‚¢ź‡‚ĶCallo-HSCT ‚š„§‚µCallo-HSCT ‘O‚É‚Ķ‰»Šw—Ć–@‚ĢŽ{s‚ą”F‚ß‚Ä‚¢‚éBTKI ‚ĢŽ”—Ć—š‚Ŗ‚ ‚éAP/BP Šś‚É‚Ķ–¢Žg—p‚ĢTKI ‚š‘I‘š‚µC‰Ā”\‚ČŒĄ‚č‘Sˆõ‚Éallo-HSCT ‚š„§‚µ‚Ä‚¢‚éB‚½‚¾‚µC‰»Šw—Ć–@‚Åallo-HSCT ‚ŖŽ{s‚Å‚«‚é‚ꂤ‚ɏ\•Ŗ•aØ‚šƒRƒ“ƒgƒ[ƒ‹‚·‚ׂ«‚Ę‚µ‚Ä‚¢‚éB‚Č‚ØCŽ”—Ć’ļR«‚ĢBP Šś‚É‘Ī‚µ‚Ä‚Ķallo-HSCT ‚Ķ„§‚³‚ź‚Ä‚¢‚Č‚¢B

yŽQl•¶Œ£z

1j Kantarjian HM, et al. Treatment of philadelphia chromosome-positive, accelerated-phase chronic myelogenous leukemia with imatinib mesylate. Clin Cancer Res. 2002 ; 8i7j : 2167-76.i3iiDivj

2j Talpaz M, et al. Imatinib induces durable hematologic and cytogenetic responses in patients with accelerated phase chronic myeloid leukemia : results of a phase 2 study. Blood. 2002 ; 99i6j : 1928-37.i3iiDivj

3j Kantarjian H, et al. Survival benefit with imatinib mesylate therapy in patients with accelerated-phase chronic myelogenous leukemia--comparison with historic experience. Cancer. 2005 ; 103 i10j : 2099-108.i3iiAj

4j le Coutre P, et al. Nilotinibiformerly AMN107j, a highly selective BCR-ABL tyrosine kinase inhibitor, is active in patients with imatinib-resistant or -intolerant accelerated-phase chronic myelogenous leukemia. Blood. 2008 ; 111i4j : 1834-9.i3iiiDivj

5j Apperley JF, et al. Dasatinib in the treatment of chronic myeloid leukemia in accelerated phase after imatinib failure : the START A trial. J Clin Oncol. 2009 ; 27i21j : 3472-9.i3iiiDivj

6j le Coutre PD, et al. Nilotinib in patients with Ph{chronic myeloid leukemia in accelerated phase following imatinib resistance or intolerance : 24-month follow-up results. Leukemia. Leukemia. 2012 ; 26 i6j : 1189-94.i3iiiAj

7j Cortes J, et al. Dasatinib induces complete hematologic and cytogenetic responses in patients with imatinib-resistant or -intolerant chronic myeloid leukemia in blast crisis. Blood. 2007 ; 109 i8j : 3207-13.i3iiiDivj

8j Nicolini FE, et al. Expanding nilotinib access in clinical trialsiENACTj, an open-label, multicenter study of oral nilotinib in adult patients with imatinib-resistant or-intolerant chronic myeloid leukemia in the accelerated phase or blast crisis. Leuk Lymphoma. 2012 ; 53i5j : 907-14.i3iiiDivj

9j Yanada M, et al. Imatinib combined chemotherapy for Philadelphia chromosome-positive acute lymphoblastic leukemia : major challenges in current practice. Leuk Lymphoma. 2006 ; 47 i9j : 1747-53.iƒŒƒrƒ…[j

10j Saussele S, et al. Allogeneic hematopoietic stem cell transplantationiallo SCTj for chronic myeloid leukemia in the imatinib era : evaluation of its impact within a subgroup of the randomized German CML Study Ⅳ. Blood. 2010 ; 115i10j : 1880-5.i2Aj

11j Baccarani M, et al. European LeukemiaNet recommendations for the management of chronic myeloid leukemia : 2013. Blood. 2013 ; 122i6j : 872-84.iƒŒƒrƒ…[j


CQ6

CMR “ž’BŒć‚ÉTKI ’†Ž~‚ĶŠ©‚ß‚ē‚ź‚é‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[4
CMR ‚Ŗ“¾‚ē‚ź‚ĈĄ‘S‚ÉTKI Ž”—Ć‚ŖI—¹‚Å‚«‚é‹K€‚ŖŠm—§‚³‚ź‚é‚Ü‚Å‚ĶC—Տ°ŽŽŒ±ˆČŠO‚ĢŽĄ—Տ°‚Å TKI ‚š’†Ž~‚·‚ׂ«‚Å‚Ķ‚Č‚¢B

y‰š ąz

IRIS ŽŽŒ±‚Ģ’·Šś¬Ń‚É‚ę‚ź‚ĪC–«ŠśCML ‚É‘Ī‚µ‚ś‚ę‚čƒCƒ}ƒ`ƒjƒuŽ”—Ć‚š‹s‚³‚ź‚½Š³ŽŅ‚Å‚ĶCŽ”—ĆŠJŽn‚ę‚萔”NŠŌ‚Ģ”NŽŸ‘ˆ«—¦‚Ķ5“ˆČ‰ŗ‚ʁCƒCƒ}ƒ`ƒjƒu“oźˆČ‘O‚Ģ10`20“‚É”ä‚ׂĒ˜–¾‚É—ĒD‚Å‚ ‚é1jB‚µ‚©‚µ‚Č‚Ŗ‚ēC×–Eˆā“`Šw“IŠ®‘S‘tŒųiCCyRj‚É“ž’B‚µ‚Ä‚¢‚銳ŽŅ‚É‘Ī‚µ‚ăCƒ}ƒ`ƒjƒuŽ”—Ć‚š’†Ž~‚·‚é‚Ę‘S—į‚ŖÄ”­‚µ‚½2jBˆź•ūC•ŖŽqˆā“`Šw“I‘tŒųm•ŖŽqˆā“`Šw“I‘å‘tŒųiMMRj‚ā•ŖŽqˆā“`Šw“IŠ®‘S‘tŒųiCMRjn‚š’·Šś‚ɈŪŽ‚µ‚Ä‚¢‚銳ŽŅ‚Ģ’†‚É‚ĶCƒCƒ}ƒ`ƒjƒu’†Ž~‚É‚ę‚Į‚Ä‚ą•ŖŽqˆā“`Šw“I‘tŒųiMRj‚ŖŽ‘±‚·‚銳ŽŅŒQ‚Ŗ‘¶Ż‚·‚邱‚Ę‚ŖŽ¦‚³‚ź3j4jC‘½Ž{Ż‹¤“ÆŽŽŒ±‚ŃCƒ}ƒ`ƒjƒu’†Ž~ŽŽŒ±iSTIM ŽŽŒ±j‚ŖŽ{s‚³‚ź‚½5jB¬l‚Å2 ”NˆČćCMR Ž‘±‚ĢCML-CP Š³ŽŅ100 l‚Ŗ“o˜^‚³‚źƒCƒ}ƒ`ƒjƒu‚Ŗ’†Ž~‚³‚ź‚½B12 ƒJŒŽˆČć‚ĢŠĻŽ@ŠśŠŌ‚š—L‚·‚é69 l‚ĢCMR ˆŪŽ—¦‚Ķ1 ”N‚Å41“C2 ”N‚Å38“‚Ę‚Č‚čC’·Šś‚ɃCƒ}ƒ`ƒjƒu‚š’†Ž~‚Å‚«‚½Š³ŽŅ‚Ģ‘¶Ż‚Ŗ–¾Šm‚É‚Č‚Į‚½5jBˆź•ūC•ŖŽqˆā“`Šw“IÄ”­Ē—į‚É‘Ī‚µƒCƒ}ƒ`ƒjƒuÄ“Š—^‚É‚ę‚čC‘S—į‚Å•ŖŽqˆā“`Šw“IŒų‰Ź‚šŽ¦‚µƒCƒ}ƒ`ƒjƒuŠ“Žó«‚Ŗ•Ū‚½‚ź‚Ä‚¢‚½B‚±‚ĢŽŽŒ±‚Å‚ĶC50 ƒJŒŽˆČć‚Ģ’·Šś‚ɃCƒ}ƒ`ƒjƒu‚Ŗ“Š—^‚³‚ź‚Ä‚¢‚邱‚ʁCSokal ƒXƒRƒA‚Ŗ’į‚¢‚±‚Ę‚ŖƒCƒ}ƒ`ƒjƒu’†Ž~‰Ā”\—vˆö‚É‹“‚°‚ē‚ź‚½B

‚Ü‚½C‘“ą‚É‚Ø‚¢‚Ä‚ąƒCƒ}ƒ`ƒjƒu“Š—^‚Ŗ’†Ž~‚³‚ź‚½Œć•ūŽ‹“I’²ø‚ŖŽĄŽ{‚³‚źCƒCƒ}ƒ`ƒjƒu“Š—^‚Ŗ’†Ž~‚³‚ź‚½43 —į‚Å47“‚Ŗ’·ŠśŠŌCMR ‚šˆŪŽ‚µ‚½B‘½•Ļ—Ź‰šĶ‚ĢŒ‹‰ŹCƒCƒ“ƒ^[ƒtƒFƒƒ“ƒæiIFNƒæj‚Ģ‘OŽ”—Ć‚ĘƒCƒ}ƒ`ƒjƒu‚Ģ‘“Š—^—Ź‚ŖÄ”R—\‘ŖˆöŽq‚Å‚ ‚Į‚½6jB

ƒCƒ}ƒ`ƒjƒu‚É”ä‚ׂĕŖŽqˆā“`Šw“IŒų‰Ź‚Ģ‚‚¢ƒ_ƒTƒ`ƒjƒu‚āƒjƒƒ`ƒjƒu‚É‚Ā‚¢‚Ä‚ĶC‘å‹K–Ķ‚Č’†Ž~ŽŽŒ±‚ĶŽ{s‚³‚ź‚Ä‚Ø‚ē‚øC\•Ŗ‚Čƒf[ƒ^‚Ŗ‚Č‚¢BCMR ‚É’B‚µ‚ĈĄ‘S‚ÉTKI ‚š’†Ž~‚·‚邽‚ß‚É‚ĶC“KŲ‚Č—Õ°ŽŽŒ±‚É‚ę‚鎊“K‚ČTKI “Š—^•ū–@‚ĢŒŸ“¢‚Ŗ•K—v‚Ę‚Č‚éBCMR ‚Ŗ“¾‚ē‚ź‚ĈĄ‘S‚ÉTKI Ž”—Ć‚ŖI—¹‚Å‚«‚é‹K€‚ŖŠm—§‚³‚ź‚é‚Ü‚Å‚ĶC—Տ°ŽŽŒ±ˆČŠO‚ÅTKI ‚š’†Ž~‚·‚é‚ׂ«‚Å‚Ķ‚Č‚¢B

yŽQl•¶Œ£z

1j Hochhaus A, et al. Six-year follow-up of patients receiving imatinib for the first-line treatment of chronic myeloid leukemia. Leukemia. 2009 ; 23i6j : 1054-61.i2Diiij

2j Goh HG, et al. Previous best responses can be re-achieved by resumption after imatinib discontinuation in patients with chronic myeloid leukemia : implication for intermittent imatinib therapy. Leuk Lymphoma. 2009 ; 50i6j : 944-51.i3iiDivj

3j Rousselot P, et al. Imatinib mesylate discontinuation in patients with chronic myelogenous leukemia in complete molecular remission for more than 2 years. Blood. 2007 ; 109i1j : 58-60.i3iiiDivj

4j Burchert A, et al. Sustained molecular response with interferon alfa maintenance after induction therapy with imatinib plus interferon alfa in patients with chronic myeloid leukemia. J Clin Oncol. 2010 ; 28 i8j : 1429-35.i3iiiDivj

5j Mahon FX, et al. Discontinuation of imatinib in patients with chronic myeloid leukaemia who have maintained complete molecular remission for at least 2 years : the prospective, multicentre Stop ImatinibiSTIMj trial. Lancet Oncol. 2010 ; 11i11j : 1029-35.i3iiiDivj

6j Takahashi N, et al. Discontinuation of imatinib in Japanese patients with CML-CP. Haematologica. 2012 ; 97i6j : 903-6.i3iDivj


CQ7

‚·‚ׂĂĢPV Ē—į‚ÉąbŒŒ‚ĶŠ©‚ß‚ē‚ź‚é‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2A
‚ ‚ē‚ä‚郊ƒXƒNŒQ‚ĢPV Š³ŽŅ‚ÉąbŒŒ‚š„§‚·‚éBąbŒŒŒć‚Ģ–Ś•WHt ’l‚Ķ45“‚Å‚ ‚éB’į—p—ŹƒAƒXƒsƒŠƒ“CƒqƒhƒƒLƒVƒEƒŒƒA“™‚É‚ę‚éRŒŒ¬”Ā—Ć–@C×–EŒø­—Ć–@•¹—pĒ—į‚Å‚ĶC‚ą‚¤­‚µ‚‚¢’li48“‚Ü‚½‚Ķ55“j‚Å‚ą‹–—e‚³‚ź‚é‚©‚ą‚µ‚ź‚Č‚¢B

y‰š ąz

—ĒD‚Ȑ¶–½—\Œć‚ŖŠś‘Ņ‚³‚ź‚éPV ‚É‘Ī‚·‚鎔—Ć‚Ģ–Ś•W‚ĶCŌŒŒ‹…‚Ģ‘‰Į‚É‚ę‚ézŠĀįŠQ‚š‰ü‘P‚µCŒŒšĒ‚āoŒŒ‚š—\–h‚·‚邱‚Ę‚Å‚ ‚éBHt ’l‚š45“–¢–ž‚Ę‚Č‚é‚ꂤƒRƒ“ƒgƒ[ƒ‹‚·‚é‚ĘŒŒšĒ”­Ē—¦‚Ŗ’į‰ŗ‚·‚é1j‚Ę‚¢‚¤•ń‚©‚ēCPV Š³ŽŅ‚ĢHt ’l‚ĶCąbŒŒ‚É‚ę‚Į‚Ä45“–¢–ž‚Ę‚Č‚é‚ꂤ‚ɃRƒ“ƒgƒ[ƒ‹‚·‚邱‚Ę‚ŖL‚­„§‚³‚ź‚Ä‚«‚½BBritish Committee for Standards in Haematology ‚ę‚č2005 ”N‚É”­•\‚³‚ź‚½ƒKƒCƒhƒ‰ƒCƒ“‚É‚Ø‚¢‚Ä‚ąCąbŒŒŒć‚Ģ–Ś•WHt ’l45“–¢–ž‚Ŗ„§‚³‚ź‚Ä‚¢‚éB‚½‚¾‚µCHt ’l45“–¢–ž‚Ę‚¢‚¤–Ś•W‚Ķ’į—p—ŹƒAƒXƒsƒŠƒ“—Ć–@‚Ŗs‚ķ‚ź‚éˆČ‘O‚Ģ•ń‚ÉŠī‚Ć‚­‚ą‚Ģ‚Å‚ ‚éBƒAƒXƒsƒŠƒ“CƒqƒhƒƒLƒVƒEƒŒƒAiHUj‚ą•¹—p‚µ‚½ąbŒŒŒć‚Ģ–Ś•WHt ’l‚ÉŠÖ‚·‚é‘O•ūŽ‹“IŒ¤‹†‚Å‚ĶC①ƒAƒXƒsƒŠƒ“CHU “™‚ōRŒŒ¬”Ā—Ć–@‚šs‚Į‚Ä‚¢‚銳ŽŅ‚Å‚ĶCHt ’l‚Ŗ55“–¢–ž‚Ģź‡‚ąC45“–¢–ž‚Ģź‡‚Ę“Æ’ö“x‚ĢŒŒšĒƒŠƒXƒNC‚Ø‚ę‚ѐ¶‘¶Š„‡‚Å‚ ‚é‚Ę‚¢‚¤•ń2j‚ʁC②Ht ’l45“–¢–ž‚š–Ś•W‚ÉąbŒŒ‚šs‚Į‚½ź‡CSŒŒŠĒįŠQ‚É‚ę‚鎀–S‚ĘŽå—vŒŒšĒ‚ĢƒŠƒXƒN‚ŖCHt ’l45`50“‚Ģź‡‚Ę”ä‚×—LˆÓ‚ÉŒø­‚·‚é‚Ę‚¢‚¤•ń3j‚ĢC‘Š”½‚·‚錋‰Ź‚Ę‚Č‚Į‚Ä‚µ‚Ü‚Į‚Ä‚¢‚éB‚Ü‚½¬”—į‚ĢŒć•ūŽ‹“IŒ¤‹†‚Å‚ĶCHt ’l‚Ŗ48“‚š’“‚¦‚é‚ĘŒŒšĒ‚Ŗ‘‰Į‚µC¶‘¶Š„‡‚Ŗ’į‰ŗ‚·‚é‚Ę•ń‚³‚ź‚Ä‚¢‚é4jB

‚±‚ź‚ē‚šŠØˆÄ‚·‚é‚ʁCŒ“‘„‚Ę‚µ‚ÄHt ’l45“‚šąbŒŒ‚Ģ–Ś•W‚Ę‚·‚ׂ«‚Å‚ ‚é‚ŖC‚ą‚¤­‚µ‚‚¢’l‚ą‹–—e‰Ā”\‚©‚ą‚µ‚ź‚Č‚¢B

yŽQl•¶Œ£z

1j Pearson TC, et al. Vascular occlusive episodes and venous haematocrit in primary proliferative polycythaemia. Lancet. 1978 ; 2i8102j : 1219-22.i3iiiCj

2j Di Nisio M, et al. European Collaboration on Low-dose Aspirin in Polycythemia VeraiECLAPj Investigators. The haematocrit and platelet target in polycythemia vera. Br J Haematol. 2007 ; 136 i2j : 249-59.i3iCj

3j Marchioli R,et al. Cardiovascular events and intensity of treatment in polycythemia vera. N Engl J Med. 2013 ; 368i1j : 22-33.i1iiiAj

4j Crisà E, et al. A retrospective study on 226 polycythemia vera patients : Impact of median hematocrit value on clinical outcomes and survival improvement with anti-thrombotic prophylaxis and non-alkylating drugs. Ann Hematol. 2010 ; 89i7j : 691-9.i3iiAj


CQ8

SŒŒŠĒƒŠƒXƒNƒtƒ@ƒNƒ^[‚š—L‚·‚éLow ƒŠƒXƒNET Ē—į‚É‘Ī‚µ‚ăAƒXƒsƒŠƒ““Š—^‚ĶŠ©‚ß‚ē‚ź‚é‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2A
Low ƒŠƒXƒNEti60 Ī–¢–žC‚©‚ĀŒŒšĒ‚ĢŠł‰‚Ŗ‚Č‚¢j‚Ģ‚Č‚©‚ŁCSŒŒŠĒƒŠƒXƒNƒtƒ@ƒNƒ^[i‹i‰ŒC‚ŒŒˆ³C‚ƒRƒŒƒXƒeƒ[ƒ‹ŒŒĒC“œ”A•aj‚Ģ‚ ‚éĒ—įCJAK2 •ĻˆŁ‚Ģ‚ ‚éĒ—į‚Å‚ĶCŒŒšĒ”­ĒƒŠƒXƒN‚š’į‰ŗ‚³‚¹‚邽‚߂ɁC RŒŒ¬”Ā—Ć–@i’į—p—ŹƒAƒXƒsƒŠƒ“75`100 mg/“śj“Š—^‚š„§‚·‚éB

y‰š ąz

High ƒŠƒXƒNEti60 ĪˆČćC‚Ü‚½‚ĶŒŒšĒ‚ĢŠł‰‚Ŗ‚ ‚éj‚Å‚ĶCƒqƒhƒƒLƒVƒEƒŒƒAiHUj‚Ę’į—p—ŹƒAƒXƒsƒŠƒ““Š—^‚ŖŒŒšĒ‚Ģ”­Ē‚š—LˆÓ‚É—}§‚µ‚½1j‚ŖCLow ƒŠƒXƒNEti60 Ī–¢–žC‚©‚ĀŒŒšĒ‚ĢŠł‰‚Ŗ‚Č‚¢j‚É‘Ī‚·‚éRŒŒ¬”Ā—Ć–@‚Ģ—L—p«‚Ķ‚±‚ź‚Ü‚Å•s–¾‚Å‚ ‚Į‚½BAlvarez-Larrán ‚ē‚ĶCLow ƒŠƒXƒNET Š³ŽŅ300 —į‚šŒć•ūŽ‹“I‚ɉšĶ‚µC–³Ž”—ĆŒo‰ßŠĻŽ@ŒQ‚ʍRŒŒ¬”Ā—Ć–@ŒQiƒAƒXƒsƒŠƒ““Š—^‚šŠÜ‚Žj‚ɂ؂Ƃ錌šĒ”­Ē•p“x‚š”äŠr‚µ‚½2jB‚±‚Ģ‰šĶ‚É‚ę‚Į‚āCLow ƒŠƒXƒNET Š³ŽŅ‚Ģ‚¤‚æCSŒŒŠĒƒŠƒXƒNƒtƒ@ƒNƒ^[i‹i‰ŒC‚ŒŒˆ³C‚ƒRƒŒƒXƒeƒ[ƒ‹ŒŒĒC“œ”A•aj‚Ģ‚ ‚銳ŽŅCJAK2 •ĻˆŁ‚Ģ‚ ‚銳ŽŅ‚É‘Ī‚·‚éRŒŒ¬”Ā—Ć–@‚ŖŒŒšĒ”­ĒƒŠƒXƒN‚š’į‰ŗ‚³‚¹‚邱‚Ę‚Ŗ–¾‚ē‚©‚Ę‚Č‚Į‚½‚±‚Ę‚ę‚čCLow ƒŠƒXƒNET ‚Å‚ąˆź•”‚ĢĒ—į‚Å‚ĶRŒŒ¬”Ā—Ć–@‚Ŗ„§‚³‚ź‚é2jBˆź•ūC‚»‚źˆČŠO‚ĢLow ƒŠƒXƒNET Š³ŽŅ‚Å‚ĶCRŒŒ¬”Ā—Ć–@‚ɏoŒŒƒŠƒXƒN‚š—½‰ķ‚·‚é—L‰v«‚ĶŒ©o‚¹‚øC“Š—^‚Ķ„§‚³‚ź‚Č‚¢B

yŽQl•¶Œ£z

1j Harrison CN, et al. United Kingdom Medical Research Council Primary Thrombocythemia 1 Study. Hydroxyurea compared with anagrelide in high-risk essential thrombocythemia. N Engl J Med. 2005 ; 353i1j : 33-45.i1iiCj

2j Alvarez-Larrán A, et al. Observation versus antiplatelet therapy as primary prophylaxis for thrombosis in low-risk essential thrombocythemia. Blood. 2010 ; 116i8j : 1205-10.i3iiCj


CQ9

Žį”NŽŅLow ƒŠƒXƒNMPN Ē—į‚É‘Ī‚µ‚ăqƒhƒƒLƒVƒEƒŒƒA‚É‚ę‚鎔—Ɖī“ü‚ĶŠ©‚ß‚ē‚ź‚é‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[4
60 Ī–¢–ž‚ĢLow ƒŠƒXƒNMPN Ē—į‚É‘Ī‚µCƒqƒhƒƒLƒVƒEƒŒƒA‚Ģ—LŒų«‚ĶŽ¦‚³‚ź‚Ä‚¢‚Č‚¢B‚Ü‚½C‹}«”’ŒŒ•a‰»‚Ü‚½‚Ķ“ńŽŸ‚Ŗ‚ń‚Ģ•p“x‚š‘‰Į‚³‚¹‚é‰Ā”\«‚Ŗ”Ū’č‚Å‚«‚Č‚¢‚½‚߁C60 Ī–¢–ž‚ĢLow ƒŠƒXƒNMPN Ē—į‚É‘Ī‚µƒqƒhƒƒLƒVƒEƒŒƒA‚É‚ę‚鎔—Ɖī“ü‚Ķ„§‚³‚ź‚Č‚¢B

y‰š ąz

MPN ‚Ģ×–E”‚šŒø­‚³‚¹‚邽‚߂ɃqƒhƒƒLƒVƒEƒŒƒAiHUj‚š‘ęˆź‘I‘š–ņ‚Ę‚µ‚Ä—p‚¢‚邱‚Ę‚Ŗ‘½‚¢BHigh ƒŠƒXƒNĒ—į‚É‘Ī‚µ‚Ä‚ĶCHU{’į—p—ŹƒAƒXƒsƒŠƒ“‚ĢŒŒšĒCoŒŒ—\–h‚É‘Ī‚·‚é—L—p«‚ŖŽ¦‚³‚ź‚Ä‚¢‚é1j‚ŖCLow ƒŠƒXƒNĒ—į‚É‘Ī‚µ‚Ä‚Ģ—L—p«‚Ķ•s–¾‚Å‚ ‚éBˆź•ūCƒuƒXƒ‹ƒtƒ@ƒ“iBUj‚Č‚Ē‚ĢƒAƒ‹ƒLƒ‹‰»Ü‚Ģ“ńŽŸ”­‚Ŗ‚ń‚Ķ‚ę‚­’m‚ē‚ź‚Ä‚¢‚é‚ŖCHU ‚Ģ“ńŽŸ”­‚Ŗ‚ń‚É‚Ā‚¢‚Ä‚ąŒœ”O‚³‚ź‚Ä‚¢‚éBET ‚É‘Ī‚·‚鉻Šw—Ć–@‚É‚ę‚é“ńŽŸ‚Ŗ‚ń‚Ę‚µ‚Ä‚ĶAML/MDS ‚Ģ‚Ł‚©NHL ‚Č‚Ē‚ĢƒŠƒ“ƒpŒnŽīį‡C”ńŒŒ‰tŽīį‡‚Ę‚µ‚Ä‚Ķ”x‚Ŗ‚ńC‘å’°‚Ŗ‚ńCt‚Ŗ‚ńCäNć÷‚Ŗ‚ńC‘O—§‘B‚Ŗ‚ń‚Č‚Ē‚³‚Ü‚“‚Ü‚ČŒÅŒ`‚Ŗ‚ń‚Ŗ•ń‚³‚ź‚Ä‚¢‚éB‚µ‚©‚µCHU ’PÜ‚É‚ę‚鎔—Ɖī“ü‚Ŗ–³Ž”—ĆŒQ‚Ę”äŠr‚µ“ńŽŸ”­‚Ŗ‚ń‚š‘‰Į‚³‚¹‚é‚©‚Ķ•s–¾‚Å‚ ‚éi11.2“ vs 7.3“j2jB‚Ü‚½CMPN ‚ĢŽ©‘RŽj‚Ę‚µ‚Ä‹}«”’ŒŒ•a‰»‚Ŗ’m‚ē‚ź‚Ä‚¢‚é‚ŖCŽ”—Ɖī“ü‚É‚ę‚č‚»‚Ģ•p“x‚Ŗ‘‰Į‚·‚é‚©”Ū‚©‚ŖŽ”—Ć‘I‘š‚šs‚¤ć‚ŏd—v‚Čƒ|ƒCƒ“ƒg‚Ę‚Č‚éB11,039 Ē—į‚Ģ‘å‹K–Ķ‚ČƒXƒEƒF[ƒfƒ“‚ĢƒRƒz[ƒgŒ¤‹†‚Å‚Ķ2.6“‚ŖAML/MDS ‚É“]‰»‚µ‚½‚ŖCHU ‚Ģ“Š—^—š‚É‚ę‚č—LˆÓ‚ɃŠƒXƒN‚Ŗ‘‰Į‚·‚邱‚Ę‚Ķ‚Č‚©‚Į‚½3jB

‰”­MPN ‚É‘Ī‚·‚éHU ’PÜ‚ĘƒRƒ“ƒgƒ[ƒ‹ŒQ‚Ģƒ‰ƒ“ƒ_ƒ€‰»”äŠrŽŽŒ±‚Ŗ‚Č‚¢‚½‚߁CHU ‚É‚ę‚é“ńŽŸ‚Ŗ‚ń‚ĢƒŠƒXƒN‚šl‚¦C‘½‚­‚Ģ—Տ°ˆć‚Ķ60 ĪˆČć‚Ü‚½‚ĶŒŒšĒ‚ĢŠł‰‚Ŗ‚ ‚éĒ—į‚š‘I‚ń‚ÅHU ‚šŽg‚Į‚Ä‚¢‚éBEuropean LeukemiaNet ‚ĶŽį”NŽŅLow ƒŠƒXƒNĒ—į‚É‘Ī‚µ‚āCHU ‚šŠÜ‚ßcytotoxic agent ‚ĢŽg—p‚šT‚¦‚é‚ꂤ„§‚µ‚Ä‚¢‚é4jBˆČć‚ę‚čŒ»Ż‚Ģ‚Ę‚±‚ėC‚ą‚µ•K—v‚Å‚ ‚ź‚Ī60 Ī–¢–ž‚Å‚Ķ”­‚Ŗ‚ń«‚Ģ‚Č‚¢IFNƒæ‚Ŗ‘ęˆź‘I‘š–ņ‚É‚Č‚é‚ʍl‚¦‚ē‚ź‚éi‚½‚¾‚µC‚ķ‚Ŗ‘‚Å‚ĶCML ˆČŠO‚ĢMPN ‚É‚ĶIFNƒæ‚Ķ•ŪŒÆ“K—pŠO‚Å‚ ‚éjB

High ƒŠƒXƒNŒQ‚ĢPV/ET ‚É‘Ī‚µ‚āC‚³‚ē‚ÉPMF ‚Å‚ĶĒóŠÉ˜a‚Ģ–Ś“I‚ÅHU ‚Ŗ•K—v‚É‚Č‚éź‡‚Ŗ‚ ‚čCĒ—į‚š‘I‘š‚µ‚ÄHU ‚šˆĄ‘S‚ÉŽg—p‚·‚é•K—v‚Ŗ‚ ‚éB

yŽQl•¶Œ£z

1j Harrison CN, et al. Hydroxyurea compared with anagrelide in high-risk essential thrombocythemia. N Engl J Med. 2005 ; 353i1j : 33-45.i1iiCj

2j Radaelli F, et al. Second malignancies in essential thrombocythemiaiETj : a retrospective analysis of 331 patients with long-term follow-up from a single institution. Hematology. 2008 ; 13i4j : 195-202.i3iiCj

3j Björkholm M, et al. Treatment-related risk factors for transformation to acute myeloid leukemia and myelodysplastic syndromes in myeloproliferative neoplasms. J Clin Oncol. 2011 ; 29i17j : 2410-5.i3iCj

4j Barbui T, et al. Philadelphia-negative classical myeloproliferative neoplasms : critical concepts and management recommendations from European LeukemiaNet. J Clin Oncol. 2011 ; 29i6j : 761-70.iƒŒƒrƒ…[j


CQ10

”DP‡•¹ET ‚É‘Ī‚µ‚Ä—¬ŽY‚šŒø­‚³‚¹‚邽‚ß‚ĢŽ”—Ɖī“ü‚ĶŠ©‚ß‚ē‚ź‚é‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2B
—¬ŽY‚šŒø‚ē‚·‚±‚Ę‚Ŗ‚Å‚«‚é‰Ā”\«‚Ŗ‚ ‚邽‚߁C­—ŹƒAƒXƒsƒŠƒ“‚É‚ę‚鎔—Ɖī“ü‚š„§‚·‚éB

y‰š ąz

”DP‡•¹ET ‚Å‚Ķ‡•¹Ē‚Ę‚µ‚Ä”DP‘Šś‚Ģ—¬ŽY‚Ŗ‘½‚­i–ń3 •Ŗ‚Ģ1jC‹H‚É•ź‘Ģ‚ĢoŒŒ‚āŒŒšĒ‚Ŗ•ń‚³‚ź‚Ä‚¢‚é‚ŖC”äŠr“I‚Ü‚ź‚Č‚½‚߁CŽ”—Ɖī“ü‚Ģ—L–³‚É‚ę‚郉ƒ“ƒ_ƒ€‰»”äŠrŽŽŒ±‚Ķ‚Č‚¢B‚µ‚©‚µ‚Č‚Ŗ‚ēC”äŠr“I‘½”—į‚ĢŒć•ūŽ‹“I‚ČŒŸ“¢‚É‚Ø‚¢‚āC­—ŹƒAƒXƒsƒŠƒ“‚ŖC”DP’†‚Ģ‡•¹Ē‚ā‘ŽY‚š—LˆÓ‚ÉŒø‚ē‚·‚Ę‚¢‚¤•ń‚Ŗ‚ ‚éB“Į‚ÉJAK2V617F ‚Ķ”DP‡•¹ET ‚É‚Ø‚¢‚臕¹Ē‚šˆų‚«‹N‚±‚·“Ę—§‚µ‚½—vˆö‚Å‚ ‚čCJAK2V617F •ĻˆŁ‚š”F‚ß‚éź‡‚ĶĻ‹É“I‚ȉī“ü‚Ŗ•K—v‚Ę‚³‚ź‚é1jB•Ź‚ĢŒć•ūŽ‹“I‚ČŒŸ“¢‚É‚Ø‚¢‚Ä‚ĶCƒCƒ“ƒ^[ƒtƒFƒƒ“ƒæiIFNƒæj‚ŖC‘ŁŽ™Ž€–S‚š—LˆÓ‚ÉŒø‚ē‚·‚Ę‚¢‚¤•ń‚Ŗ‚ ‚éB“Į‚ÉJAK2V617F •ĻˆŁ‚Ķ—¬ŽY‚š‚«‚½‚·“Ę—§‚µ‚½—\Œć•s—ĒˆöŽq‚Å‚ ‚čCIFNƒæ‚ÅŒŒ¬”Ā”‚šŒø‚ē‚·‚±‚Ę‚É‚ę‚č‡•¹Ē‚š‰ń”š‚Å‚«‚é‰Ā”\«‚Ŗ‚ ‚é2jB”DP‡•¹ET 400 —į‚ĢƒVƒXƒeƒ}ƒeƒBƒbƒNƒŒƒrƒ…[‚Å‚ĶƒŠƒXƒN‚š–ā‚ķ‚øC”DP‡•¹ET ‚É‘Ī‚µ‚Ä‚Ģ­—ŹƒAƒXƒsƒŠƒ“‚Ģ—L—p«‚É‚Ā‚¢‚ÄŒ¾‹y‚µ‚Ä‚¢‚é3jB‚µ‚©‚µ‚Č‚Ŗ‚ēCŒŒ¬”Ā”‚Ŗ100 –œ‚š’“‚¦‚éĒ—į‚Å‚ĶoŒŒŒXŒü‚šŽ¦‚·‚½‚ߏ­—ŹƒAƒXƒsƒŠƒ“‚Ķ‹ÖŠõ‚Ę‚Č‚éB‚Ü‚½CoŽY‚Ģ1`2 T‘O‚É‚ĶƒAƒXƒsƒŠƒ“‚š’†Ž~‚µCoŽYŒćoŒŒ‚Ŗ‚Č‚¢‚±‚Ę‚šŠm”F‚µ‚½ŒćCƒAƒXƒsƒŠƒ“‚šÄŠJ‚µ6 TŠŌ‚ĶŒp‘±‚·‚邱‚Ę‚Ŗ„§‚³‚ź‚Ä‚¢‚é3jB‚Ü‚½CJAK2V617F •ĻˆŁ‚š—L‚·‚éHigh ƒŠƒXƒNĒ—į‚É‘Ī‚µ‚Ä‚Ķ­—ŹƒAƒXƒsƒŠƒ“{IFNƒæi•ŪŒÆ“K—pŠOj‚É‚ę‚鎔—Ɖī“ü‚ąl—¶‚³‚ź‚éB

yŽQl•¶Œ£z

1j Passamonti, et al. Aspirin in pregnant patients with essential thrombocythemia : a retrospective analysis of 129 pregnancies. J Thromb Haemost. 2010 ; 8i2j : 411-3.i3iCj

2j Melillo L, et al. Outcome of 122 pregnancies in essential thrombocythemia patients : A report from the Italian registry. Am J Hematol. 2009 ; 84i10j : 636-40.i3iCj

3j Griesshammer M, et al. Management of Philadelphia negative chronic myeloproliferative disorders in pregnancy. Blood Rev. 2008 ; 22i5j : 235-45.iƒŒƒrƒ…[j


CQ11

PMF ‚É‘Ī‚µ‚Ä“ÆŽķ‘¢ŒŒŠ²×–EˆŚA‚ĶŠ©‚ß‚ē‚ź‚é‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2B
Žį”NCHigh ƒŠƒXƒNPMF ‚É‘Ī‚·‚é‘¢ŒŒŠ²×–EˆŚA‚ĶŽ”—ĆƒIƒvƒVƒ‡ƒ“‚Ę‚µ‚Đ„§‚³‚ź‚éB

y‰š ąz

PMF ‚ĢŖŽ”‚Ŗ–]‚߂鎔—Ć‚Ķallo-HSCT ‚Å‚ ‚čC“KŲ‚Čƒhƒi[‚Ŗ‘¶Ż‚·‚éŽį”NŽŅ‚Å‚ĶˆŚA—Ć–@‚ŖL‚­s‚ķ‚ź‚Ä‚¢‚éB‚µ‚©‚µ‚Č‚Ŗ‚ēCallo-HSCT ‚ÉŠÖ‚ķ‚鎔—ĆŠÖ˜AŽ€–Sitherapy-related mortalityFTRMj‚Ķ­‚Č‚­‚Č‚¢i30`50“jB

ÅV‚Ģ‘½Ž{Ż‘O•ūŽ‹“I‹¤“ÆŒ¤‹†‚Ŗ‰p‘‚Ęƒtƒ‰ƒ“ƒX‚©‚ē•ń‚³‚ź‚Ä‚¢‚éB‰p‘‚Ģ•ń‚Å‚ĶC51 —į‚ĢPMF ‚É‘Ī‚µ‚čœ‘”j‰ó“IˆŚA‚Ü‚½‚Ķœ‘”ń”j‰ó“IˆŚAireduced-intensity stem cell transplantationFRISTj‚šs‚¢C3 ”N‘S¶‘¶Š„‡iOSj‚Ķ‚»‚ź‚¼‚ź44“‚Ę33“‚ŁC3 ”NTRM ‚Ķ‚»‚ź‚¼‚ź41“‚Ę32“‚Å‚ ‚Į‚½BÄ”­‚Ķ‚»‚ź‚¼‚ź15“‚Ę46“‚ɁCextensive cGVHD ‚š‚»‚ź‚¼‚ź30“‚Ę35“‚É”F‚ß‚Ä‚¢‚é1jBˆź•ūCƒtƒ‰ƒ“ƒX‚Ģ•ń‚Å‚Ķ147 —į‚ĢMFiPMF 53“ , Secondary MF 47“j‚É‘Ī‚µ‚čœ‘”j‰ó“IˆŚA‚Ü‚½‚ĶRIST ‚šs‚¢C4 ”NOS ‚Ķ39“‚ŁC4 ”N–³‘ˆ«¶‘¶Š„‡iPFSj‚Ķ32“C4 ”N”ńÄ”­Ž€–SŠ„‡inon-relapse mortalityFNRMj‚Ŗ39“‚Å‚ ‚Į‚½2jBTRM ‚šŒø‚ē‚·‚±‚Ę‚ŖŠś‘Ņ‚³‚ź‚éRIST ‚ÅŒŒ‰tŠw“IÄ”­‚Ŗ‘½‚¢ŒXŒü‚Ŗ‚ ‚邽‚߁CŒ»Ż‚Ü‚Å‚Ģ‚Ę‚±‚ėPMF ‚É‘Ī‚·‚éRIST ‚Ģ—L—p«‚Ķ–¾‚ē‚©‚Å‚Č‚¢B

ƒ‰ƒ“ƒ_ƒ€‰»”äŠrŽŽŒ±‚É‚ę‚é‚ʁCallo-HSCT ‚Ģ•K—v‚ȏĒ—į‚Ę“KŲ‚ČˆŚAŽžŠśCˆŚA‘Oˆ’u‚Ķ–¾‚ē‚©‚Ę‚³‚ź‚Ä‚Ø‚ē‚øCŒ»Žž“_‚Å‚Ķ‘˜_‚Ģ•\7 ‚ÉŽ¦‚µ‚½—\Œć—\‘Ŗƒ‚ƒfƒ‹‚É‚ę‚č—\Œć•s—ĒiHigh ‚Ü‚½‚ĶInt-2 ƒŠƒXƒNj‚Ę”»’f‚³‚źC“KŲ‚Čƒhƒi[‚Ŗ‘¶Ż‚·‚éŽį”NŽŅ‚Å‚ĶCŖŽ”“I‚ČŽ”—Ć‚Å‚ ‚éallo-HSCT ‚ŖŽ”—ĆƒIƒvƒVƒ‡ƒ“‚Ę‚µ‚Đ„§‚³‚ź‚éB

yŽQl•¶Œ£z

1j Stewart WA, et al. The role of allogeneic SCT in primary myelofibrosis : a British Society for Blood and Marrow Transplantation study. Bone Marrow Transplant. 2010 ; 45i11j : 1587-93.i3iiiAj

2j Robin M, et al. Allogeneic haematopoietic stem cell transplantation for myelofibrosis : a report of the Société Française de Greffe de Moelle et de Thérapie CellulaireiSFGM-TCj. Br J Haematol. 2011 ; 152i3j : 331-9.i3iiiAj


’Ē‰ĮCQ

High ƒŠƒXƒN–PMF Š³ŽŅ‚Ģ–ņ•Ø—Ć–@‚Ģ‘ęˆź‘I‘š–ņ‚Ķ‰½‚©B
–IPSS ƒŠƒXƒN•Ŗ—Ž‚Å’†ŠŌ-ⅡA‚Ü‚½‚Ķ‚ƒŠƒXƒN


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[1
ƒ‹ƒLƒ\ƒŠƒ`ƒjƒu‚ĶAIPSS ƒŠƒXƒN•Ŗ—Ž‚Å’†ŠŌ-ⅡA‚Ü‚½‚Ķ‚ƒŠƒXƒNMF Š³ŽŅ‚ĢäBŽīA‘SgĒói‘~įyŠ“A‘SgŒ‘‘ÓŠ“A“Š¾Aœ’É‚Č‚Ēj‚Ģ‰ü‘PŒų‰Ź‚š—L‚·‚éB

y‰š ąz

2 ‚Ā‚ĢƒtƒF[ƒY3 ŽŽŒ±‚©‚ēAMFŠ³ŽŅ‚É‘Ī‚·‚郋ƒLƒ\ƒŠƒ`ƒjƒu‚ĢŽg—p‚ĶAƒvƒ‰ƒZƒ{iCOMFORT-ⅠŽŽŒ±j‚Ü‚½‚ĶŠł‘¶‚ĢŽ”—Ɓibest available therapy: BATjiCOMFORT-ⅡŽŽŒ±j‚Ę”äŠr‚µ‚āAäBŽīA‘SgĒói‘~įyŠ“A‘SgŒ‘‘ÓŠ“A“Š¾Aœ’É‚Č‚Ēj‚Ģ‰ü‘P‚š‚ą‚½‚ē‚·‚±‚Ę‚ŖŽ¦‚³‚ź‚Ä‚¢‚é1j2jB—¼ŽŽŒ±‚Ģ’·ŠśƒtƒHƒ[ƒAƒbƒv‚É‚ę‚é‚ʁAƒ‹ƒLƒ\ƒŠƒ`ƒjƒuŽ”—Ć‚ŖŒp‘±‚³‚ź‚½Ē—į‚Å‚ĶAäBŽīA‘SgĒó‚Ģ‰ü‘PŒų‰Ź‚Ķ’·ŠśŠŌ‚É‚ķ‚½‚Į‚ĈŪŽ‚³‚ź‚Ä‚¢‚é3j4jB‚±‚ź‚ē‚ĢŽŽŒ±‚Ķ¶‘¶‚šƒGƒ“ƒhƒ|ƒCƒ“ƒg‚É‚µ‚½‚ą‚Ģ‚Å‚Ķ‚Č‚¢‚ŖA’·ŠśƒtƒHƒ[ƒAƒbƒv‚ĢŠĻŽ@‚É‚ę‚é‚ʁAƒ‹ƒLƒ\ƒŠƒ`ƒjƒuŒQ‚É‚Ø‚¢‚摶ŠśŠŌ‚Ģ—LˆÓ‚ȉ„’·‚Ŗ”F‚ß‚ē‚ź‚Ä‚¢‚éB‚Č‚ØCOMFORT ŽŽŒ±‚ĶAIPSS‚ĢIntermediate-2 ƒŠƒXƒN‚Ü‚½‚ĶHigh ƒŠƒXƒN‚©‚ĀŒŒ¬”Ā”†10 –œŒĀ/ƒŹl‚ĢMFŠ³ŽŅ‚š‘ĪŪ‚Ę‚µ‚½‚ą‚Ģ‚Å‚ ‚é‚ŖAŒŒ‹…Œø­‚Ŗ‚ ‚鏼‡‚É‚ąA­—Ź‚©‚ē‚Ģƒ‹ƒLƒ\ƒŠƒ`ƒjƒu“Š—^‚É‚ę‚éˆĄ‘S«‚Ę—LŒų«‚Ŗ•ń‚³‚ź‚Ä‚¢‚é5jB

ƒ‹ƒLƒ\ƒŠƒ`ƒjƒu‚Ķ‚·‚®‚ź‚½—Տ°Œų‰Ź‚šŽ¦‚·‚ą‚Ģ‚ĢAŽīį‡ƒNƒ[ƒ“‚ĢŒø­Œų‰Ź‚Ķ‹Ķ‚©‚Å‚ ‚邱‚Ę‚Ŗ’m‚ē‚ź‚Ä‚¢‚éB‚»‚Ģ‚½‚߁A—\Œć—\‘Ŗƒ‚ƒfƒ‹‚É‚ę‚č—\Œć•s—Ē‚Ę”»’f‚³‚źA“KŲ‚Čƒhƒi[‚Ŗ‘¶Ż‚·‚éŽį”NŽŅ‚É‘Ī‚µ‚Ä‚ĶAallo-HSCT ‚Ģ‰Ā”\«‚š‚Ü‚øl—¶‚·‚ׂ«‚Å‚ ‚éB

yŽQl•¶Œ£z

1j Verstovsek S, et al. A double-blind, placebo-controlled trial of ruxolitinib for myelofibrosis. N Engl J Med. 2012;366:799-807.i1iCj

2j Harrison C, et al. JAK inhibition with ruxolitinib versus best available therapy for myelofibrosis. N Engl J Med. 2012;366:787-98.i1iiCj

3j Verstovsek S, et al. Efficacy, safety and survival with ruxolitinib in patients with myelofibrosis: results of a median 2-year follow-up of COMFORT-I. Haematologica. 2013;98:1865-71.i3iiAj

4j Cervantes F, et al. Three-year efficacy, safety, and survival findings from COMFORT-II, a phase 3 study comparing ruxolitinib with best available therapy for myelofibrosis. Blood. 2013;122:4047-53.i3iiAj

5j Talpaz M, et al. Interim analysis of safety and efficacy of ruxolitinib in patients with myelofibrosis and low platelet counts. J Hematol Oncol. 2013 Oct 29;6(1):81.i3iiCj


5
  –«ƒŠƒ“ƒp«”’ŒŒ•a/¬ƒŠƒ“ƒp‹…«ƒŠƒ“ƒpŽī
ichronic lymphocytic leukemiaFCLL/small lymphocytic lymphomaFSLLj

Ÿ‘˜_

–«ƒŠƒ“ƒp«”’ŒŒ•aichronic lymphocytic leukemiaFCLLj‚Ķ’Pˆź‚ȏ¬Œ^‰~Œ`‚©‚ēŒy“x‚ĢˆŁŒ^‚šŽ‚ĀB ƒŠƒ“ƒp‹…‚ĢŽīį‡‚ŁCCD5 ‚ĘCD23 ‚Ģ”­Œ»‚Ŗ‚Ż‚ē‚źC“ś–{‚Å‚Ķ‹H‚ČŽīį‡‚Å‚ ‚éB¬ƒŠƒ“ƒp‹…«ƒŠƒ“ƒpŽīismall lymphocytic lymphomaFSLLj‚Ķ––½ŒŒ‚āœ‘‚Ö‚ĢZ‚Ŗ‚Č‚¢CLL ‚Ę“Æˆź‚Ģ×–E‚ĢŽīį‡‚Ę’č‹`‚³‚ź1jC•aŠś‚āŽ”—Ć‚Ģl‚¦•ū‚Ķ’įˆ««“xB ×–EƒŠƒ“ƒpŽī‚Ę‚µ‚Ĉµ‚¤‚Ģ‚Å‘¼e‚šŽQl‚É‚·‚éB

‘½‚­‚ĶŠÉ™‚ČŒo‰ß‚šŽ¦‚·‚ŖCˆź•”‚ɐis‚Ŗ‘¬‚­C—\Œć•s—Ē‚Č‚ą‚Ģ‚Ŗ‚Ż‚ē‚ź‚éB•aŠś•Ŗ—Ž‚É‚Ä•aŠś‚šŒˆ’肵CŽ”—ĆŠJŽn‹K€‚ɏ€‚¶‚ÄŽ”—Ć‚šŽĄŽ{‚·‚éB‰¢•Ä‚Å‚ĶƒŠƒcƒLƒVƒ}ƒuiRjiCLL ‚Å‚Ķ‘“ą“K‰žŠOj‚š•¹—p‚·‚éƒtƒ‹ƒ_ƒ‰ƒrƒ“iFLUj‚šŠÜ‚ŽŽ”—Ć‚Ŗ•W€Ž”—Ć‚Å‚ ‚é‚ŖCõF‘Ģ17p Œ‡Žø‚ĢĒ—į‚ĶŽ”—Ć’ļR«‚Å—\Œć•s—Ē‚Å‚ ‚é2j3jB

1D•aŠś•Ŗ—Ž

Ž”—Ć•ūj‚ĢŒˆ’č‚É•K{‚ŁC•Ä‘‚Å‚Ķ‰ü’łRai •Ŗ—ŽC‰¢B‚Å‚ĶBinet •Ŗ—Ži•\1j2j3j‚ŖŽg—p‚³‚ź‚éBfŽ@ŠŒ©‚Ę•nŒŒCŒŒ¬”ĀŒø­‚¾‚ƂŐf’f‚µCCT ‚Č‚Ē‚Ģ‰ę‘œŠŒ©‚Ķ—p‚¢‚Č‚¢B

•\1-1@‰ü’łRai ‚Ģ•aŠś•Ŗ—Ž•\
‰ü’łRai •Ŗ—Ž Rai •Ŗ—Ž•aŠś •Ŗ—Ž‹K€
’įƒŠƒXƒN 0 ––½ŒŒƒŠƒ“ƒp‹…„15,000/ƒŹL–
{
œ‘ƒŠƒ“ƒp‹…„ 40“
’†ŠŌƒŠƒXƒN •aŠś0{ƒŠƒ“ƒpßŽī’Æ
•aŠś0`Ⅰ
{ŠĢŽīCäBŽīi‚Ē‚æ‚ē‚©‚Ü‚½‚Ķ—¼•ūj
‚ƒŠƒXƒN •aŠś0`Ⅱ
{•nŒŒiHbƒ11 g/dL ‚Ü‚½‚ĶHtƒ33“j
•aŠś0`Ⅲ{ŒŒ¬”Āƒ10–œ /ƒŹL

–ƒŠƒ“ƒp‹…”‚ÉŠÖ‚µ‚Ä‚ĶCŒ»Ż‚ĢCLL f’f‹K€‚ĶB ƒŠƒ“ƒp‹…”‚Ŗ5,000/ƒŹL ˆČć‚Ę‚Č‚Į‚Ä‚Ø‚čC•aŠś•Ŗ—Ž‚ĘˆŁ‚Č‚Į‚Ä‚¢‚éB

•\1-2@Binet ‚Ģ•aŠś•Ŗ—Ž
•aŠś •Ŗ—Ž‹K€
A Hb†10 g/dL
{ŒŒ¬”Ā†10 –œ/ƒŹL
{ƒŠƒ“ƒp—ĢˆęŽī‘å‚Ŗ2 ƒJŠˆČ‰ŗ
B Hb†10 g/dL
{ŒŒ¬”Ā†10 –œ/ƒŹL
{ƒŠƒ“ƒp—ĢˆęŽī‘å‚Ŗ3ƒJŠˆČć
C Hbƒ10 g/dL
‚Ü‚½‚ĶŒŒ¬”Āƒ10 –œ/ƒŹLL
ƒŠƒ“ƒpßŽī‘å—Ģˆę”‚Ķ‹K’肵‚Č‚¢

ƒŠƒ“ƒpß—Ģˆę‚Ķ①“Ŗčņ•”C②ćüā|C③‘lŒa•”C④äB‘ŸC⑤ŠĢ‘Ÿ‚Ģ5 —Ģˆęi—¼‘¤‚Å‚ą1—Ģˆę‚Ę•]‰æ‚·‚éjBg‘ĢfŽ@‚Ģ‚Ż‚ĢŠŒ©‚Å‚ ‚éB

2D—\ŒćˆöŽq

1j•aŠś•Ŗ—Ž

¶‘¶’†‰›’l‚Ķ‰ü’łRai •Ŗ—Ž’įƒŠƒXƒN‚Ķ10 ”NˆČćC’†ŠŌƒŠƒXƒN‚Ķ7 ”NC‚ƒŠƒXƒN‚Ķ1.5`3 ”NC Binet •Ŗ—Ž‚Ģ•aŠśA Šś‚Ķ10 ”NˆČćCB Šś‚Ķ7 ”NCC Šś‚Ķ1.5`2.5 ”N‚Å‚ ‚é2jB

2j‘¼‚Ģ—\Œć•s—ĒˆöŽq

①–ʉuƒOƒƒuƒŠƒ“d½iIgVHjˆā“`Žq•ĻˆŁ‰A«C②CD38 ”­Œ»C③zeta-associated protein of 70kDaiZAP-70j”­Œ»‚šŽ¦‚·‚ą‚Ģ‚Ķ—\Œć•s—Ē‚ŁC‚±‚ź‚ē‚ĢĒ—į‚É—\Œć•s—Ē‚ĢõF‘ĢˆŁķm11qiATM Ąj‚Ę17pip53 Ąj‚ĢŒ‡Žø‚ĢõF‘ĢˆŁķn‚šŽ¦‚·‚ą‚Ģ‚Ŗ‘½‚¢B‚»‚Ģ‘¼C‚—īC’j«C‚Ń‚Ü‚ń«œ‘ZC’Z‚¢ƒŠƒ“ƒp‹…”{‰ĮŽžŠŌCKi67 ‚”­Œ»CŒŒ“ƒ`ƒ~ƒWƒ“ƒLƒi[ƒ[CƒĄ2 ƒ~ƒNƒƒOƒƒuƒŠ ƒ“‚ā‰Ā—n«CD23CTNFƒæ‚’lClipoprotein lipase ‚”­Œ»CmicroRNA ”­Œ»•ĻˆŁCŽ”—Ć”½‰ž«‚Ŗˆ« ‚¢C‚ą‚µ‚­‚Ķ’ZŠśŠŌ‚Å‚ĢÄ”­‚Å‚ ‚é4j`7jB

3DŽ”—Ć

CLL ‚ĶŒo‰ß‚Ģ’·‚¢Ž¾Š³‚Å‚ ‚邽‚߁CŽ”—ĆŠÖ˜AŽ€–S‚Ķ”š‚Æ‚é‚ׂ«‚Å‚ ‚éBTd‚ČŽ”—Ć•ū–@‚Ģ‘I‘š‚ĢŒŸ“¢‚Ŗ•K{‚Å‚ ‚čC‚»‚ĢŠT—Ŗ‚šˆČ‰ŗ‚ÉŽ¦‚·B

  1. ‰ü’łRai •Ŗ—Ž‚Ģ’įƒŠƒXƒNiRai •Ŗ—Ž•aŠś0 Šśj‚āBinet •Ŗ—Ž‚Ģ•aŠśA ‚ĢŠ³ŽŅ‚Ķ‘Šś‚ĢŽ”—Ć‚Ķ‘S¶‘¶ŠśŠŌ‚š‰„’·‚µ‚Č‚¢‚½‚ߌo‰ßŠĻŽ@‚·‚éB
  2. ‰ü’łRai •Ŗ—Ž‚Ģ’†ŠŌƒŠƒXƒNiRai •Ŗ—Ž•aŠśⅠCⅡŠśj‚āBinet •Ŗ—Ž‚Ģ•aŠśB ‚Ģ‘½‚­‚ĢŠ³ŽŅ‚Ķis‚ŖŠÉ™‚Å‚ ‚é‚Ģ‚ŁCŒo‰ßŠĻŽ@‚·‚éB
  3. ‰ü’łRai •Ŗ—Ž‚Ģ‚ƒŠƒXƒNiRai •Ŗ—Ž•aŠśⅢCⅣŠśj‚ ‚é‚¢‚ĶBinet •Ŗ—Ž‚ĢC Šś‚Ü‚½‚Ķis«B Šś‚ĢŠ³ŽŅ‚ĶŽ”—Ć‚Ģ‘ĪŪ‚Å‚ ‚éBCLL ‚ĶŽ”–ü¢“ļ‚Å‚ ‚é‚Ŗ’·Šś¶‘¶‚Ŗ‰Ā”\‚Å‚ ‚čC‚Ü‚½‚—īŽŅ‚Ŗ‘½‚¢‚½‚߁Cˆź•”‚ĢŽį”NŽŅ‚šœ‚«Ž”–ü‚š–ŚŽw‚·‚ę‚čĒóŠÉ˜a‚ā”’ŒŒ•a‚Ģ•aØ‚ĢƒRƒ“ƒgƒ[ƒ‹‚Ŗ–Ś“I‚Å‚ ‚éB
  4. Ž”—ĆŠJŽn‹K€i•\2, 3jFŽ”—Ć‚ĢŠJŽnŽžŠś‚ĶƒŠƒ“ƒp‹…‚Ģā‘Ī”‚Å‚Ķ‚Č‚­CƒKƒCƒhƒ‰ƒCƒ“3j`5j8j‚šŽQl‚É‚·‚éB
•\2@ESMO ƒKƒCƒhƒ‰ƒCƒ“2j‚ÉŽ¦‚³‚ź‚½Šˆ“®«•a‘Ō
  1. 1jB Ēó
    1. ”­”MF38Ž‚ę‚č‚‚¢Œ“ˆö•s–¾‚Ģ”­”M
    2. QŠ¾FQ‹ļiƒ}ƒbƒgƒŒƒXˆČŠO‚ĢŠ|‚Æ•z’cCƒV[ƒc‚Č‚Ē‚šŠÜ‚ŽCQŠŌ’…‚ĶŠÜ‚Ü‚Č‚¢j‚š•Ļ‚¦‚Č‚Æ‚ź‚Ī‚Č‚ē‚Č‚¢‚Ł‚Ē‚Ģ‚ø‚Ō”G‚ź‚É‚Č‚éŠ¾
    3. ‘ĢdŒø­Ff’f‘O‚Ģ6 ƒJŒŽ‚É’Źķ‘Ģd‚Ģ10“‚š’“‚·Œ“ˆö•s–¾‚Ģ‘ĢdŒø­
  2. 2jŽ©ŒČ–ʉu‚É‚ę‚ē‚Č‚¢ŒŒ‹…Œø­
  3. 3jƒŠƒ“ƒpßŽī’ƁCŠĢäBŽī‚É‚ę‚éĒó‚āĒŒó
  4. 4j•›t”ēŽæƒXƒeƒƒCƒhƒzƒ‹ƒ‚ƒ“‚É”½‰ž‚Ģˆ«‚¢Ž©ŒČ–ʉu«•nŒŒ‚āŒŒ¬”ĀŒø­
•\3@International Workshop on Chronic Lymphocytic Leukemia4j8j‚ÅŽ¦‚³‚ź‚½Ž”—ĆŠJŽn‹K€
ˆČ‰ŗ‚Ģ€–Ś‚Ģ‚¢‚ø‚ź‚©‚ÉŠY“–‚·‚ź‚ĪCŠˆ“®«iactive diseasej‚Ę‚µCŽ”—Ć‚šl—¶‚·‚éB
  1. 1jis«‚Ģœ‘‹@”\’į‰ŗ‚É‚ę‚é•nŒŒ‚āŒŒ¬”ĀŒø­‚ĢisEˆ«‰»
  2. 2j¶˜]œ‹|‰ŗ6 cm ˆČć‚ĢäBŽīCis«‚Ü‚½‚ĶĒŒó«‚ĢäBŽī
  3. 3j’·Œa10 cm ˆČć‚ĢƒŠƒ“ƒpß‰ņCis«‚Ü‚½‚ĶĒŒó«‚ĢƒŠƒ“ƒpßŽī’Æ
  4. 4j2 ƒJŒŽˆČ“ą‚É50“‚š’“‚¦‚éis«ƒŠƒ“ƒp‹…‘‰ĮC6 ƒJŒŽˆČ‰ŗ‚ĢƒŠƒ“ƒp‹…”{‰ĮŽžŠŌ
  5. 5j•›t”ēŽæƒXƒeƒƒCƒh‚ā‘¼‚Ģ•W€Ž”—Ć‚É”½‰ž‚Ģˆ«‚¢Ž©ŒČ–ʉu«•nŒŒ‚āŒŒ¬”ĀŒø­Ē
  6. 6jCLL ‚É‹Nˆö‚·‚éˆČ‰ŗ‚Ģ‚¢‚ø‚ź‚©‚ĢĒó‚Ģ‚ ‚é‚Ę‚«
    1. Œø—Ź‚É‚ę‚ē‚Č‚¢‰ß‹Ž6 ƒJŒŽˆČ“ą‚Ģ10“ˆČć‚Ģ‘ĢdŒø­
    2. ˜J“­‚ā“śķ¶Šˆ‚Ŗ¢“ļ‚Å‚ ‚éiECOG PS 2 ˆČćj‚ĢŒ‘‘ÓŠ“
    3. Š“õĒ‚ĢŠŒ©‚Č‚µ‚É2 TŠŌˆČć‘±‚­38ŽˆČć‚Ģ”­”M
    4. Š“õĒ’„Œó‚Ģ‚Č‚¢QŠ¾
4DŽ”—ĆŒų‰Ź”»’č

Ž”—ĆŒų‰Ź”»’č3j‚Ķ•\4 ‚É‚ę‚Į‚ÄŽĄŽ{‚·‚éB

•\4@Ž”—ĆŒų‰Ź”»’č3j

Š®‘S‘tŒųicomplete responseFCRj

ˆČ‰ŗ‚ĢŠī€‚š‚·‚ׂĖž‚½‚·ó‘Ō‚ŖC3 ƒJŒŽˆČćŒp‘±‚·‚邱‚Ę

  1. ––½ŒŒ’†‚ɃNƒ[ƒiƒ‹‚ČB ƒŠƒ“ƒp‹…‚Ŗ‚Č‚¢‚±‚Ę
  2. Œa1.5 cm ˆČć‚ĢƒŠƒ“ƒpß‚Ŗ‚Č‚¢‚±‚Ę
  3. fŽ@‚ÅŠĢäBŽī‚Ŗ‚Č‚¢‚±‚Ę
  4. Į–Ր«‚ĢĒó‚Ŗ‚Č‚¢‚±‚Ę
  5. ŒŒ‹…‚ŖˆČ‰ŗ‚ĢšŒ
    1. 1jD’†‹…„1,500/ƒŹL
    2. 2jŒŒ¬”Ā„10 –œ/ƒŹL
    3. 3j—AŒŒ‚µ‚Č‚¢ó‹µ‚Ńwƒ‚ƒOƒƒrƒ“„11.0 g/dL

•”•Ŗ‘tŒųipartial responseFPRj

ˆČ‰ŗ‚ĢŠī€‚š­‚Č‚­‚Ę‚ą2 ‚ĀˆČć–ž‚½‚·ó‘Ō‚ŖC2 ƒJŒŽˆČćŒp‘±‚·‚邱‚Ę

  1. ––½ŒŒ’†‚ɃNƒ[ƒiƒ‹‚ČB ƒŠƒ“ƒp‹…‚Ŗ50“ˆČćŒø­‚·‚邱‚Ę
  2. ƒŠƒ“ƒpß‚Ŗ50“ˆČćŒø­
  3. ŠĢäBŽī‚Ŗ50“ˆČćŒø­
  4. ŒŒ‹…‚ŖˆČ‰ŗ‚ĢšŒ
    1. 1jD’†‹…„ 1,500/ƒŹL ‚Ü‚½‚ĶŽ”—Ć‘O‚ę‚č50“ˆČć‚Ģ‰ü‘P
    2. 2jŒŒ¬”Ā„ 10 –œ/ƒŹL ‚Ü‚½‚ĶŽ”—Ć‘O‚ę‚č50“ˆČć‚Ģ‰ü‘P
    3. 3j—AŒŒ‚µ‚Č‚¢ó‹µ‚Ńwƒ‚ƒOƒƒrƒ“„ 11.0 g/dLC‚Ü‚½‚ĶŽ”—Ć‘O‚ę‚č50“ˆČć‚Ģ‰ü‘P

isiprogressionj

ˆČ‰ŗ‚ĢŠī€‚š­‚Č‚­‚Ę‚ą1 ‚ĀˆČć–ž‚½‚·ó‘Ō

  1. ƒŠƒ“ƒpßŽī’Æ
    1. 1jŒa1.5 cm ˆČć‚ĢV‚½‚ČƒŠƒ“ƒpßŽī’ƁCV‚½‚ČŠĢäBŽīC‘ŸŠķZ
    2. 2jÅ‘åŒa50“ˆČć‚Ģ‘‰ĮCŒa1 `1.5 cm ‚ĢƒŠƒ“ƒpß‚Å‚Ķ50“‚Ģ‘‰ĮC
      @‚Ü‚½‚ĶŒa1.5 cm ˆČćC1.5 cm ˆČć‚ĢƒŠƒ“ƒpß‚Å‚Ķ’·Œa2.0 cm ˆČć‚ɂȂ邱‚Ę
    3. 3j‘½”­‚µ‚Ä‚¢‚郊ƒ“ƒpß‚ĢŒa‚Ģ˜a‚Ģ50“ˆČć‚Ģ‘‘å
  2. ŠĢ‘ŸCäB‘Ÿ‚ĢƒTƒCƒY‚Ģ50“ˆČć‚Ģ‘‘å
  3. ––½ŒŒ’†ƒŠƒ“ƒp‹…”‚Ģ50“ˆČć‚Ģ‘‘å‚Ü‚½‚ĶB ƒŠƒ“ƒp‹…”5,000/ƒŹL ˆČć
  4. Richter ĒŒóŒQ‚Ģ‚ꂤ‚Č‘B‚Ŗ‘¬‚¢Žīį‡‚Ö‚ĢŒ`Žæ“]Š·i‰Ā”\‚ČŒĄ‚čCƒŠƒ“ƒpß“™‚Ģ¶ŒŸ‚ÅŠm”F‚·‚éj
  5. CLL ‚ĘŠÖ˜A‚Ģ‚ ‚錌‹…Œø­‚ĢoŒ»

ˆĄ’čistable diseasej

CR ‚āPR ‚É’B‚¹‚øCis‚É‚ą‚ ‚½‚ē‚Č‚¢ź‡

yŽQl•¶Œ£z

1j Muller-Hermalink HK, et al. Chronic lymphocytic leykaemia/small lymphocytic lymphoma. In : Swerdlow SH, et al. Editors. World Health Organization classification of tumors. Lyon : IARC Press ; 2008. pp180-2.

2j Eichhorst B, et al ; ESMO Guidelines Working Group. Chronic lymphocytic leukaemia : ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2010 ; 21 Suppl 5 : v162-4.iƒKƒCƒhƒ‰ƒCƒ“j

3j NCCN clinical practice guidelins in oncology. Non-Hodgkinfs lymphoma. version 2. 2012iƒKƒCƒhƒ‰ƒCƒ“j

4j Hallek M, et al ; International Workshop on Chronic Lymphocytic Leukemia. Guidelines for the diagnosis and treatment of chronic lymphocytic leukemia : a report from the International Workshop on Chronic Lymphocytic Leukemia updating the National Cancer Institute-Working Group 1996 guidelines. Blood. 2008 ; 111i12j : 5446-56.iƒKƒCƒhƒ‰ƒCƒ“j

5j Oscier D, et al ; Guidelines Working Group of the UK CLL Forum. British Committee for Standards in Haematology. Guidelines on the diagnosis and management of chronic lymphocytic leukaemia. Br J Haematol. 2004 ; 125i3j : 294-317.iƒKƒCƒhƒ‰ƒCƒ“j

6j Gribben JG. How I treat CLL up front. Blood. 2010 ; 115i2j : 187-97.iƒŒƒrƒ…[j

7j Gribben JG, et al. Update on therapy of chronic lymphocytic leukemia. J Clin Oncol. 2011 ; 29i5j : 544-50.iƒŒƒrƒ…[j

8j Cheson BD, et al. National Cancer Institute-sponsored Working Group guidelines for chronic lymphocytic leukemia : revised guidelines for diagnosis and treatment. Blood. 1996 ; 87i12j : 4990-7.iƒKƒCƒhƒ‰ƒCƒ“j



ŸƒAƒ‹ƒSƒŠƒYƒ€

i¦jCQ”Ō†iƒsƒ“ƒNF•”•Ŗj‚šƒNƒŠƒbƒN‚·‚é‚ʁC‰šą‰ę–Ź‚ÖˆŚ“®‚µ‚Ü‚·

–«ƒŠƒ“ƒp«”’ŒŒ•a/¬ƒŠƒ“ƒp‹…«ƒŠƒ“ƒpŽī‚ĢƒAƒ‹ƒSƒŠƒYƒ€ CQ1 CQ2 CQ4

FC —Ć–@:ƒtƒ‹ƒ_ƒ‰ƒrƒ“+ƒVƒNƒƒzƒXƒtƒ@ƒ~ƒh—Ć–@, F —Ć–@:ƒtƒ‹ƒ_ƒ‰ƒrƒ“—Ć–@, BSC:best supportive care

CLL ‚Ģf’f‚Ŗ‚Č‚³‚ź‚½ź‡C‘Šś‚ĢCLLm‘˜_‚Ģ•\2 ‚ÉŽ¦‚·Šˆ“®«‚Ģ•a‘Ō‚Ŗ‚Č‚¢Binet •aŠś•Ŗ—ŽA Šś‚ĘB ŠśC‚Ø‚ę‚Ń–³Ēó‚Ģ‰ü’łRai •Ŗ—Ž•aŠś’įE’†ŠŌƒŠƒXƒNiRai •Ŗ—Ž•aŠś0 ŠśCⅠŠśCⅡŠśjnŠ³ŽŅ‚ĶCŒo‰ßŠĻŽ@‚·‚邱‚Ę‚Ŗ„§‚³‚ź‚éiCQ1jBŠˆ“®«’„Œó‚Ŗ‚Ż‚ē‚ź‚½‚čCisŠś‚É‚Č‚Į‚½ź‡‚Ķ‘½Ü•¹—p‰»Šw—Ć–@C–ʉu‰»Šw—Ć–@‚Ŗ‰Ā”\‚©‚Ē‚¤‚©‚š•]‰æ‚µ‚āC‰Ā”\‚Å‚ ‚ź‚Īƒtƒ‹ƒ_ƒ‰ƒrƒ“iFLUj‚šŠÜ‚Ž‰»Šw—Ć–@mFLU{ƒVƒNƒƒzƒXƒtƒ@ƒ~ƒhiCPAj—Ć–@FFC —Ć–@nC‚Ø‚ę‚Ń‚»‚ź‚ɃŠƒcƒLƒVƒ}ƒuiRj‚Ģ•¹—p—Ć–@iR ‚ĶCLL ‚Å‚Ķ‘“ą“K‰žŠOj‚šŽĄŽ{‚·‚éiCQ2jB‚±‚ź‚ē‚ĢŽ”—Ć‚É”½‰ž‚Ŗ‚ ‚鏼‡‚Å‚ąCõF‘Ģ17p Œ‡Žø‚Ģ‚ ‚鏼‡‚ĶC—\Œć•s—Ē‚Å‚ ‚邱‚Ę‚Ŗ–¾‚ē‚©‚Å‚ ‚邽‚߁C“ÆŽķ‘¢ŒŒŠ²×–EˆŚAiallogeneic hematopoietic stem cell transplantation : allo-HSCTj‚šl—¶‚·‚éiCQ3, CQ4jB‘½Ü•¹—p‰»Šw—Ć–@‚Ŗ•¹‘¶Ž¾Š³‚Č‚Ē‚Ģ‘¶Ż‚Å•s‰Ā”\‚Å‚ ‚ź‚ĪCFLU ’P“Ę—Ć–@iF —Ć–@j‚ā‘¼‚ĢƒAƒ‹ƒLƒ‹‰»Ü‚Č‚Ē‚Ģ’P“Ę—Ć–@‚āŒø—Ź‚Ģ‘½Ü•¹—p‰»Šw—Ć–@‚šl—¶‚·‚éiCQ2jBÄ”­‚āŽ”—Ć’ļR«‚šŽ¦‚·ź‡‚Ķ‹~‰‡—Ć–@‚šŽĄŽ{ŒćC•”•Ŗ‘tŒųŽį”NŽŅ‚āõF‘Ģ17p Œ‡Žø‚š—L‚·‚éĒ—į‚Ķallo-HSCT ‚šl—¶‚·‚éB‚Ü‚½CÄ”­E“ļŽ”—į‚Å‚Ķ‰¢•Ä‚Å‚Ķalemtuzmab —Ć–@‚Ŗ•W€Ž”—Ć‚Ę‚µ‚ÄŽĄŽ{‚³‚ź‚Ä‚¢‚éBalemtuzmab —Ć–@‚Ķ—\Œć•s—Ē‚ĢõF‘Ģ17p Œ‡Žø—į‚É‚ą—LŒų«‚ŖŽ¦‚³‚ź‚Ä‚¢‚é‚ŖC‘“ą–¢³”F–ņ‚Å‚ ‚éiCQ3jBŽ©‰Ę‘¢ŒŒŠ²×–EˆŚA•¹—p‘å—Ź‰»Šw—Ć–@‚ĶCƒ‰ƒ“ƒ_ƒ€‰»”äŠrŽŽŒ±‚Å‘S¶‘¶ŠśŠŌ‚Ģ‰„’·‚šŽ¦‚³‚Č‚¢‚½‚ߐ„§‚³‚ź‚Č‚¢Ballo-HSCT ‚ĶCŽį”NŽŅ‚ŁC‹~‰‡—Ć–@‚É”½‰ž‚Ŗ‚ ‚鏼‡‚É‚Ķl—¶‚·‚éiCQ4jB



CQ1

‚Ē‚Ģ‚ꂤ‚ČCLL Š³ŽŅ‚šŽ”—Ć‚·‚ׂ«‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[1
Šˆ“®«‚Ģ•a‘Ō‚Ŗ‚Č‚¢Binet •aŠś•Ŗ—ŽA ‚ĘBC‚Ø‚ę‚Ń–³Ēó‚Ģ‰ü’łRai •Ŗ—Ž’įƒŠƒXƒN‚Ø‚ę‚Ń’†ŠŌƒŠƒXƒNiRai •Ŗ—Ž•aŠś0`ⅡŠśjŠ³ŽŅ‚ĶCŽ”—Ć‚šŠJŽn‚µ‚Ä‚ą¶‘¶‚ĢŒüć‚É‚ĶŠń—^‚µ‚Č‚¢‚½‚߁CŒo‰ßŠĻŽ@‚·‚邱‚Ę‚Ŗ„§‚³‚ź‚éB

„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2A
isŠśiŠˆ“®«•a‘Ō‚Ģ‚ ‚éBinet A ‚ĘB ŠśCBinet C ŠśCĒó‚Ģ‚ ‚éRai •Ŗ—Ž•aŠś0`ⅡŠśCRai •Ŗ—Ž•aŠśⅢ`ⅣŠśj‚ĢŠ³ŽŅ‚ĶŽ”—Ć‚šŠJŽn‚·‚邱‚Ę‚šl—¶‚·‚éB

y‰š ąz

ŠCŠO‚Ģ—Տ°ŽŽŒ±1j2j‚Ęƒƒ^ƒAƒiƒŠƒVƒX3j‚ĢŒ‹‰Ź‚©‚ēCŠˆ“®«•a‘Ō‚Ŗ‚Č‚¢Binet •aŠś•Ŗ—ŽA ‚ĘBC‚Ø‚ę‚Ń–³Ēó‚Ģ‰ü’łRai •Ŗ—Ž’įƒŠƒXƒN‚Ø‚ę‚Ń’†ŠŌƒŠƒXƒNiRai •Ŗ—Ž•aŠś0`ⅡŠśj‚ĢŽžŠś‚©‚ēŽ”—Ć‚šŠJŽn‚µ‚Ä‚ąC10 ”N‘S¶‘¶Š„‡‚ŖŽ”—ĆŒQ44“C–¢Ž”—ĆŒQ47“‚ʐ¶‘¶ŠśŠŌ‚Ģ‰„’·‚Ķ“¾‚ē‚ź‚Č‚©‚Į‚½B‚±‚ź‚ē‚Ģƒ‰ƒ“ƒ_ƒ€‰»”äŠrŽŽŒ±‚Ķ‘“ą–¢³”F–ņ‚Å‚ ‚échlorambucil ‚Ę‚Ģ”äŠrŽŽŒ±‚Å‚ ‚é‚ŖC‚·‚ׂĂĢŽŽŒ±‚Ő¶‘¶ŠśŠŌ‚Ģ‰„’·‚šŽ¦‚³‚Č‚©‚Į‚½B‚Ü‚½CŽ”—ĆŒQ‚Ģ“ńŽŸ‚Ŗ‚ń”­¶—¦‚Ŗćø‚µ‚½4jB

‚±‚ź‚ē‚Ģƒf[ƒ^‚Ķ‚·‚ׂāCŒ»Ż‚Ģ•W€Ž”—Ć–ņ‚Ģƒtƒ‹ƒ_ƒ‰ƒrƒ“iFLUj‚É‚ę‚錋‰Ź‚Å‚Ķ‚Č‚¢‚½‚߁C‚±‚Ģó‘Ō‚ɂ؂Ƃ鎔—Ć‚Ģ—LŒų«‚šŒŸŲ‚·‚é—Տ°ŽŽŒ±‚ŖŒ»Żis’†‚Å‚ ‚éB‚±‚ĢŒ‹‰Ź‚Ŗ–¾‚ē‚©‚É‚Č‚é‚܂ŁC‚±‚Ģ•aŠś‚Å‚ĢŽ”—Ć‚Ķ—Տ°ŽŽŒ±‚Å‚Ģ‚ŻŽĄŽ{‚³‚ź‚é‚ׂ«‚Å‚ ‚é5jB

International Workshop on Chronic Lymphocytic Leukemia ‚ĢŽ”—ĆŠJŽn‹K€m‘˜_‚Ģ•\3 5j6jn‚ŖŽ¦‚³‚ź‚Ä‚¢‚é‚ŖC‘¼‚Ģ’įˆ««“xƒŠƒ“ƒpŽī‚Ę‚ĶˆŁ‚Č‚čCisŠś‚ĢŠ³ŽŅ‚ŁC–³Ž”—ĆŒo‰ßŠĻŽ@‚ĘŽ”—ĆŒQ‚Ę‚Ģƒ‰ƒ“ƒ_ƒ€‰»”äŠrŽŽŒ±‚ŖŽĄŽ{‚³‚ź‚½‚ķ‚Æ‚Å‚Ķ‚Č‚¢B‚µ‚©‚µ‚Ł‚Ę‚ń‚Ē‚ĢŠ³ŽŅ‚Ŗ—LĒó‚Å‚ ‚čCŽ”—Ć‚¹‚ø‚ÉŒo‰ßŠĻŽ@‚š‚·‚邱‚Ę‚Ķ•s“KŲ‚ʍl‚¦‚ē‚ź‚邽‚߁CŽ”—Ć‚šl—¶‚·‚ׂ«‚Å‚ ‚éB

yŽQl•¶Œ£z

1j Dighiero G, et al. Chlorambucil in indolent chronic lymphocytic leukemia. French Cooperative Group on Chronic Lymphocytic Leukemia. N Engl J Med. 1998 ; 338i21j : 1506-14.i1iiAj

2j Shustik C, et al. Treatment of early chronic lymphocytic leukemia : intermittent chlorambucil versus observation. Hematol Oncol. 1988 ; 6i1j : 7-12.i1iiAj

3j Chemotherapeutic options in chronic lymphocytic leukemia : a meta-analysis of the randomized trials. CLL Trialistsf Collaborative Group. J Natl Cancer Inst. 1999 ; 91i10j : 861-8.i1iiAj

4j Gribben JG, et al. Update on therapy of chronic lymphocytic leukemia. J Clin Oncol. 2011 ; 29i5j : 544-50.iƒŒƒrƒ…[j

5j Hallek M, et al. International Workshop on Chronic Lymphocytic Leukemia. Guidelines for the diagnosis and treatment of chronic lymphocytic leukemia : a report from the International Workshop on Chronic Lymphocytic Leukemia updating the National Cancer Institute-Working Group 1996 guidelines. Blood. 2008 ; 111i12j : 5446-56.iƒKƒCƒhƒ‰ƒCƒ“j

6j Cheson BD, et al. National Cancer Institute-sponsored Working Group guidelines for chronic lymphocytic leukemia : revised guidelines for diagnosis and treatment. Blood. 1996 ; 87i12j : 4990-7.iƒKƒCƒhƒ‰ƒCƒ“j


CQ2

‰”­isŠśCLL ‚ĢÅ“K‚ČŽ”—Ć‚Ķ‰½‚ŖŠ©‚ß‚ē‚ź‚é‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2A
70 Ī–¢–ž‚Ü‚½‚Ķ‚»‚źˆČć‚Ģ”N—ī‚Å‚ą•¹‘¶Ē‚Č‚Ē‚Ģ•]‰æ‚š‚µ‚ĉĀ”\‚Å‚ ‚ź‚ĪC–ʉu‰»Šw—Ć–@‚Ŗ„§‚³‚ź‚éB

„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2A
‚—īŽŅ‚Ü‚½‚ĶŽį”NŽŅ‚Å•¹‘¶Ē‚Ģ‚ ‚鏼‡‚ĶŽ”—Ć‹­“x‚šŒø‚¶‚½Ž”—Ć‚Ŗ„§‚³‚ź‚éB

y‰š ąz

NCCN ƒKƒCƒhƒ‰ƒCƒ“i2012Cver.2j‚Å‚Ķ•¹‘¶Ē‚Č‚Ē‚Ģ•]‰æ‚ĶCumulative Illness Rating Scale iCIRSjC‚»‚Ģ‘¼Comprehensive Geriatric assessment ‚Ę‚µ‚ÄMini-Mental State Examination iMMSEjC1.5-item Geriatric Depression Scale, Barthel Index, instrumental activities of daily living iIADLsj‚š—p‚¢‚é1jB•]‰æŒćC‰Ā”\‚Å‚ ‚ź‚Ī–ʉu‰»Šw—Ć–@‚šl—¶‚·‚éBƒtƒ‹ƒ_ƒ‰ƒrƒ“iFLUj—Ć–@ŒQ‚Ķchlorambucil —Ć–@i‘“ą–¢³”F–ņj‚ę‚č‘S¶‘¶Š„‡‚Ŗ—ĒD‚Å‚ ‚邽‚ߎ”—Ć–ņ‚Ę‚µ‚Ä‚ĶFLU ‚šŽg—p‚·‚é2jB

FLU ‚ĘƒVƒNƒƒzƒXƒtƒ@ƒ~ƒhiCPAj‚Ģ•¹—p—Ć–@‚ĶFLU ’P“Ę—Ć–@ŒQ‚Ę”ä‚ׂ摶ŠśŠŌ‚Ķ•Ļ‚ķ‚ē‚Č‚©‚Į‚½‚ŖC‘tŒųŠ„‡‚ā–³‘ˆ«¶‘¶ŠśŠŌiPFSj‚š‰ü‘P‚³‚¹3j`5jChealth-related quality of lifeiQOLj‚Ģ‘ˆ«‚Ķ‚Ż‚ē‚ź‚Č‚©‚Į‚½6j‚±‚Ę‚©‚ē•W€Ž”—Ć‚Ģˆź‚Ā‚Ę‚³‚ź‚Ä‚¢‚邽‚߁CŽĄŽ{‰Ā”\‚Å‚ ‚ź‚Īl—¶‚·‚éB

ƒNƒ‰ƒhƒŠƒrƒ“‚ĘCPA ‚Ģ•¹—p—Ć–@7j‚āƒxƒ“ƒ_ƒ€ƒXƒ`ƒ“8j‚ą—LŒų‚Å‚ ‚éB

ƒŠƒcƒLƒVƒ}ƒuiRj‚ĶCLL ×–E•\–Ź‚ĢCD20 ”­Œ»‚ĢŽć‚¢‚±‚ʁCŒŒŠĒ“ą‚ĢŽīį‡—Ź‚Ŗ‘½‚¢‚½‚ß‘‚­‚ɍR‘Ģ‚ŖĮŽø‚·‚邱‚Ę‚Č‚Ē‚ŖŒ“ˆö‚Å’PÜ‚Å‚Ģ—LŒų«‚ĶŒĄ‚ē‚ź‚Ä‚¢‚é9j10jBR •¹—p‰»Šw—Ć–@‚ĶŽ”—Ć”½‰ž«‚ŖÅ‚ą‚‚¢Ž”—Ć–@‚ŁC•W€Ž”—Ć‚Ęl‚¦‚ē‚ź‚é‚ŖC–{–M‚Å‚ĶCR ‚Ķ‘“ą“K‰žŠO‚Å‚ ‚čC‚Ü‚½Œ»Žž“_‚Å‚Ķ‚·‚ׂĂĢCLL Š³ŽŅ‚Ģ‘S¶‘¶ŠśŠŌiOSj‚š‰„’·‚³‚¹‚é‚Ę‚¢‚¤ƒf[ƒ^‚Ķ‚Č‚¢11jB

‚—īŽŅ‚Ģ–¾Šm‚Č’č‹`‚Ķ‚Č‚¢‚ŖC70 ĪˆČći‚ą‚µ‚­‚Ķ65 ĪˆČćj‚ʍl‚¦‚ē‚ź‚Ä‚¢‚éB’Źķ—Ź‚ĢŽ”—Ć‚Ŗ‰Ā”\‚©‚Ē‚¤‚©‚ĶƒKƒCƒhƒ‰ƒCƒ“1j‚ɏ]‚Į‚Ä‚¢‚邱‚Ę‚Ŗ‘½‚¢B‚Ü‚½C‚—īŽŅŠ³ŽŅ‚¾‚Æ‚š‘ĪŪ‚Ę‚µ‚½ƒGƒrƒfƒ“ƒX‚Ģ‚‚¢—Տ°ŽŽŒ±‚Ķ‚Č‚¢B

”N—ī‚Ģ§ŒĄ‚Ŗ‚Č‚¢‘å‹K–Ķ—Տ°ŽŽŒ±‚Å‚ĶC”N—ī‚É‚ę‚莔—Ɛ¬Ń‚Ģ·‚Ķ‚Ż‚ē‚ź‚Č‚¢4j5j11jB‚µ‚©‚µCŽĄŪ‚Ķ‚—īŽŅ‚ā•¹‘¶Ē‚Ŗ‚ ‚銳ŽŅ‚Å‚Ķ’Źķ—Ź‚Å‚Ģ•W€Ž”—Ć‚šŽĄŽ{‚·‚邱‚Ę‚Ķ¢“ļ‚Å‚ ‚čCŠ³ŽŅŒĀX‚Ģó‹µ‚š‚ę‚­”cˆ¬‚µCŽ”—Ć•ū–@‚Ģ‘I‘š‚š‚·‚ׂ«‚Å‚ ‚éB

NCCN ƒKƒCƒhƒ‰ƒCƒ“i2012, ver.2j‚É‚Ķchlorambucil}RCR ’P“Ę—Ć–@CR •¹—pƒxƒ“ƒ_ƒ€ƒXƒ`ƒ“—Ć–@CCPA{PSL } RCalemtuzumabCFLU ’P“Ę—Ć–@CR •¹—pFLU —Ć–@CƒNƒ‰ƒhƒŠƒrƒ“’P“Ę—Ć–@CR •¹—pƒNƒ‰ƒhƒŠƒrƒ“—Ć–@‚Č‚Ē‚ŖŽ”—Ć•ū–@‚Ę‚µ‚Ä‹“‚°‚ē‚ź‚Ä‚¢‚éB65 ĪˆČć‚ĢŠ³ŽŅ‚š‘ĪŪ‚É‚µ‚½FLU ‚Ęchlorambucil ‚Ģƒ‰ƒ“ƒ_ƒ€‰»”äŠrŽŽŒ±‚ÅFLU ‚Ģ—DˆŹ«‚ĶŲ–¾‚³‚ź‚ø12jC‚—īŽŅ‚É‘Ī‚·‚éFLU{CPA •¹—p—Ć–@‚ĶFLU ’P“Ę—Ć–@‚É”äŠr‚µ‚Ä‘tŒųŠ„‡‚Ķ‚‚¢‚ŖC¶‘¶ŠśŠŌ‚Ķ‰„’·‚¹‚øCœ‘—}§‚Č‚Ē‚Ģ—LŠQŽ–Ū‚Ģ•p“x‚Ŗ‚‚¢5j‚½‚߁C‰¢•Ä‚Å‚Ķchlorambucil ’P“Ę—Ć–@‚Ŗ•W€Ž”—Ć‚Ęl‚¦‚ē‚ź‚Ä‚¢‚éB‘“ą‚Å‚Ķchlorambucil ‚ŖŽg—p‚Å‚«‚Č‚¢‚½‚߁CFLU ‚š’†S‚Ę‚·‚鎔—Ć‚ŖŽĄŽ{‚³‚ź‚é‚ŖC‚»‚Ģ—p—Ź‚ĶŒˆ’č‚³‚ź‚Ä‚¢‚Č‚¢B‚Ü‚½R ‚Ģ•¹—p‚ÉŠÖ‚µ‚Ä‚ą–¾Šm‚ČƒGƒrƒfƒ“ƒX‚Ķ‚Č‚¢B

FLU ‚ĶŽå‚ɐt”rŸ•‚Å‚ ‚邱‚ʁC”N—ī‚Ę‚Ę‚ą‚ɐt‹@”\C‚Ę‚­‚ɃNƒŒƒAƒ`ƒjƒ“ƒNƒŠƒAƒ‰ƒ“ƒX‚Ŗ–ˆ”N1“‚ø‚Ā’į‰ŗ‚·‚邽‚߁C‚—īŽŅ‚Å‚ĶFLU ‚Ģ‘ćŽÓ‚ɉe‹æ‚šŽó‚Æ‚ā‚·‚¢‚±‚Ę‚Č‚Ē‚©‚ēCFLUCCPA ‚šŒø—Ź‚µ‚½—Տ°ŽŽŒ±‚Ŗ‚¢‚­‚Ā‚©‚ ‚čCŒø—Ź‚š‚·‚邱‚Ę‚ÅŠ“õĒ‚šŠÜ‚ß‚½—LŠQŽ–Ū‚ŖŒyŒø‚µC‘tŒ÷Š„‡‚Ķ•Ļ‚ķ‚ē‚ø—L—p‚Å‚ ‚é‚Ę•ń‚³‚ź‚Ä‚¢‚é‚ŖC‚¢‚ø‚ź‚ąƒGƒrƒfƒ“ƒXƒŒƒxƒ‹‚Ķ’į‚¢13j`15jB

’1j R ‚Ķ‘“ą‚É‚Ø‚¢‚Ä‚ĶCLL ‚Ģ•ŪŒÆ“K—pŠO‚Å‚ ‚é‚ŖCCLL ‚Ę“Æ‚¶Ž¾Š³‚Å‚ ‚éSLL ‚Ķ’įˆ««“xB ×–EƒŠƒ“ƒpŽī‚Å‚ ‚čC‚±‚Ģź‡‚Ķ‘“ą‚É‚Ø‚¢‚Ä‚ą•ŪŒÆ“K—p‚Ę‚Č‚Į‚Ä‚¢‚éB

’2j chlorambucil, alemtuzumab ‚Ķ‘“ą–¢³”FCƒxƒ“ƒ_ƒ€ƒXƒ`ƒ“CƒNƒ‰ƒhƒŠƒrƒ“‚ĶCLL ‚É‘Ī‚µ ‚č‘“ą“K‰žŠO‚Å‚ ‚éB

yŽQl•¶Œ£z

1j Salvi F, et al. A manual of guidelines to score the modified cumulative illness rating scale and its validation in acute hospitalized elderly patients. J Am Geriatr Soc. 2008 ; 56i10j : 1926-31.iƒKƒCƒhƒ‰ƒCƒ“j

2j Rai KR. Long-term survival analysis of the North American Intergroup Study C9011 comparing fludarabine iFj and chlorambucil in previously untreated patients with chronic lymphocytic leukemia iCLLjBlood. 2009 ; 114 : 224iabstract #536ji1iiAj

3j Eichhorst BF, et al. German CLL Study Group. Fludarabine plus cyclophosphamide versus fludarabine alone in first-line therapy of younger patients with chronic lymphocytic leukemia. Blood. 2006 ; 107i3j : 885-91.i1iiAj

4j Flinn IW, et al. Phase Ⅲ trial of fludarabine plus cyclophosphamide compared with fludarabine for patients with previously untreated chronic lymphocytic leukemia : US Intergroup Trial E2997. J Clin Oncol. 2007 ; 25i7j : 793-8.i1iiDiiij

5j Catovsky D, et al. UK National Cancer Research InstituteiNCRIj Haematological Oncology Clinical Studies Group ; NCRI Chronic Lymphocytic Leukaemia Working Group. Assessment of fludarabine plus cyclophosphamide for patients with chronic lymphocytic leukaemiaithe LRF CLL4 Trialj : a randomised controlled trial. Lancet. 2007 ; 370i9583j : 230-9.i1iiA/1iiDiiij

6j Eichhorst BF, et al. German CLL Study Group. Health-related quality of life in younger patients with chronic lymphocytic leukemia treated with fludarabine plus cyclophosphamide or fludarabine alone for first-line therapy : a study by the German CLL Study Group. J Clin Oncol. 2007 ; 25i13j : 1722-31.i1iiCj

7j Robak T, et al. Comparison of cladribine plus cyclophosphamide with fludarabine plus cyclophosphamide as first-line therapy for chronic lymphocytic leukemia : a phase Ⅲ randomized study by the Polish Adult Leukemia GroupiPALG-CLL3 Studyj. J Clin Oncol. 2010 ; 28i11j : 1863-9.i1iiDivj

8j Knauf WU, et al. Phase Ⅲ randomized study of bendamustine compared with chlorambucil in previously untreated patients with chronic lymphocytic leukemia. J Clin Oncol. 2009 ; 27i26j : 4378-84.i1iiDiiij

9j Byrd JC, et al. Rituximab using a thrice weekly dosing schedule in B-cell chronic lymphocytic leukemia and small lymphocytic lymphoma demonstrates clinical activity and acceptable toxicity. J Clin Oncol. 2001 ; 19i8j : 2153-64.i3iiiDivj

10j OfBrien SM, et al. Rituximab dose-escalation trial in chronic lymphocytic leukemia. J Clin Oncol. 2001 ; 19i8j : 2165-70.i3iiiDivj

11j Hallek M, et al. International Group of Investigators ; German Chronic Lymphocytic Leukaemia Study Group. Addition of rituximab to fludarabine and cyclophosphamide in patients with chronic lymphocytic leukaemia : a randomised, open-label, phase 3 trial. Lancet. 2010 ; 376i9747j : 1164-74.i1iiDiiij

12j Eichhorst BF, et al. German CLL Study GroupiGCLLSGj. First-line therapy with fludarabine compared with chlorambucil does not result in a major benefit for elderly patients with advanced chronic lymphocytic leukemia. Blood. 2009 ; 114i16j : 3382-91.i1iiAj

13j Robertson LE, et al. A 3-day schedule of fludarabine in previously treated chronic lymphocytic leukemia. Leukemia. 1995 ; 9i9j : 1444-9.i3iiiAj

14j Marotta G, et al. Low-dose fludarabine and cyclophosphamide in elderly patients with B-cell chronic lymphocytic leukemia refractory to conventional therapy. Haematologica. 2000 ; 85i12j : 1268-70.i3iiiDivj

15j Forconi F, et al. Low-dose oral fludarabine plus cyclophosphamide in elderly patients with untreated and relapsed or refractory chronic lymphocytic Leukaemia. Hematol Oncol. 2008 ; 26i4j : 247-51.i3iiiAj


CQ3

Ä”­E“ļŽ”«CLL ‚ĢŽ”—Ć‚Ķ‰½‚ŖŠ©‚ß‚ē‚ź‚é‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2A
ƒtƒ‹ƒ_ƒ‰ƒrƒ“‚āƒAƒ‹ƒLƒ‹‰»–ņ’PÜŽ”—Ć‚Ģź‡‚Ķƒtƒ‹ƒ_ƒ‰ƒrƒ“{ƒVƒNƒƒzƒXƒtƒ@ƒ~ƒh•¹—p—Ć–@‚ą‚µ‚­‚Ķ–ʉu‰»Šw—Ć–@‚šl—¶‚·‚éB

„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2A
‰»Šw—Ć–@’P“ĘŽ”—ĆŒć‚ĢÄ”­‚É‚Ķ–ʉu‰»Šw—Ć–@‚šl—¶‚·‚éB

„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[1
õF‘Ģ17p Œ‡Žø‚āp53 ˆā“`ŽqˆŁķ‚Ŗ‚ ‚鏼‡‚Ķ’Źķ‚Ģ‰»Šw—Ć–@‚É‘Ī‚µ‚Ä“ļŽ”«‚Å‚ ‚é‚ŖCalemtuzumab ‚Ŗ—LŒų‚Å‚ ‚éialemtuzumab ‚Ķ‘“ą–¢³”FjB

y‰š ąz

‰”­Š³ŽŅ‚ĢŽ”—Ć‚Ę“Æ—l‚ɁC‚—īŽŅ‚ā‘ŸŠķįŠQ‚Ŗ‚ ‚鏼‡‚ĶŒø—Ź‚Č‚Ē‚šl—¶‚·‚éBƒtƒ‹ƒ_ƒ‰ƒrƒ“iFLUj‚āƒAƒ‹ƒLƒ‹‰»–ņ’PÜŽ”—Ć‚ÅÄ”­CŽ”—Ć’ļR«‚Ģź‡‚ĶFLU{ƒVƒNƒƒzƒXƒtƒ@ƒ~ƒhiCPAj•¹—p—Ć–@1j‚āƒŠƒcƒLƒVƒ}ƒuiRj•¹—pFLU{CPA —Ć–@2j‚Ŗ—LŒų‚ŁC‚Ü‚½FLU{CPA •¹—p—Ć–@Œć‚Å‚ąR •¹—pFLU{CPA —Ć–@‚Ŗ—LŒų‚Å‚ ‚é3j‚Ģ‚ŁC‚±‚ź‚ē‚ĢŽ”—Ć‚šl—¶‚·‚éBFLU Ž”—Ć’ļR«EÄ”­‚Ģź‡Calemtuzumabi‘“ą–¢³”Fj4j‚āR •¹—pƒxƒ“ƒ_ƒ€ƒXƒ`ƒ“—Ć–@5j‚Ŗ—LŒų‚Å‚ ‚éBÄ”­‚Ü‚½‚Ķ“ļŽ”«‚ĢCLL ‚É‘Ī‚µ‚ÄR ‚Ę‚ĶˆŁ‚Č‚éƒGƒsƒg[ƒv‚š”FŽÆ‚·‚éV‹KRCD20 R‘Ģ–ņƒIƒtƒ@ƒcƒ€ƒ}ƒu‚Ŗ‘“ą³”F‚³‚ź‚½B‚»‚Ģ‚½‚߁Cƒtƒ‹ƒ_ƒ‰ƒrƒ“‚āalemtuzumab ’ļR«‚Ģź‡‚¾‚Æ‚Å‚Č‚­CR Ž”—ĆŒć‚Å‚ąƒIƒtƒ@ƒcƒ€ƒ}ƒu‚Ŗ—LŒų‚Č‚±‚Ę‚Ŗ‚ ‚é6jB

FLU Ž”—ĆŒć‚ĢÄ”­‚āŽ”—Ć’ļR«‚ŁC õF‘Ģ17p Œ‡Žø‚āp53 ˆā“`ŽqˆŁķ‚Ŗ‚ ‚鏼‡‚Ķalemtuzumab ‚Ŗ—LŒųi‘tŒųŠ„‡40“j‚Å‚ ‚é7j8jB‚»‚Ģ‘¼CƒGƒrƒfƒ“ƒXƒŒƒxƒ‹‚Ķ’į‚¢‚ŖCCHOP —Ć–@iCPA, DXR, VCR, PSLj‚āhyper-CVAD —Ć–@iCPA, VCR, DXR, DEXj‚Č‚ĒƒŠƒ“ƒpŽī‹~‰‡—Ć–@‚Ę“Æ—l‚ČŽ”—Ć‚ŖNCCN ƒKƒCƒhƒ‰ƒCƒ“i2012, ver.2j‚É‚ą‹LŚ‚³‚ź‚Ä‚¢‚éB‚Ü‚½‚±‚ź‚ē‚ĢŽ”—Ć‚ÉR ‚Ŗ•¹—p‚³‚ź‚邱‚Ę‚Ŗ‚ ‚éB

yŽQl•¶Œ£z

1j OfBrien SM, et al. Results of the fludarabine and cyclophosphamide combination regimen in chronic lymphocytic leukemia. J Clin Oncol. 2001 ; 19i5j : 1414-20.i3iiiDiiij

2j Robak T, et al. Rituximab plus fludarabine and cyclophosphamide prolongs progression-free survival compared with fludarabine and cyclophosphamide alone in previously treated chronic lymphocytic leukemia. J Clin Oncol. 2010 ; 28i10j : 1756-65.i1iiDiiij

3j Wierda W, et al. Chemoimmunotherapy with fludarabine, cyclophosphamide, and rituximab for relapsed and refractory chronic lymphocytic leukemia. J Clin Oncol. 2005 ; 23i18j : 4070-8.i3iiiDivj

4j Elter T, et al. Fludarabine plus alemtuzumab versus fludarabine alone in patients with previously treated chronic lymphocytic leukaemia : a randomized phase 3 trial. Lancet Oncol. 2012 ; 12 i3j : 1204-13.i1iiDiiij

5j Fischer K, et al. Bendamustine combined with rituximab in patients with relapsed and/or refractory chronic lymphocytic leukemia : A multicenter phase ‡U trial of the German Chronic Lymphocytic Leukemia Study Group. J Clin Oncol. 2011 ; 29i26j : 3559-66.i3iiiDivj

6j Wierda WG, et al. Hx-CD20-406 Study Investigators. Ofatumumab is active in patients with fludarabinerefractory CLL irrespective of prior rituximab : results from the phase 2 international study. Blood. 2011 ; 118i19j : 5126-9.i3iiiDivj

7j Lozanski G, et al. Alemtuzumab is an effective therapy for chronic lymphocytic leukemia with p53 mutations and deletions. Blood. 2004 ; 103i9j : 3278-81.i3iiiDivj

8j Stilgenbauer S, et al. German Chronic Lymphocytic Leukemia Study Group. Subcutaneous alemtuzumab in fludarabine-refractory chronic lymphocytic leukemia : clinical results and prognostic marker analyses from the CLL2H study of the German Chronic Lymphocytic Leukemia Study Group. J Clin Oncol. 2009 ; 27i24j : 3994-4001.i3iiiDivj


CQ4

CLL ‚É‘Ī‚·‚é‘¢ŒŒŠ²×–EˆŚA‚ĶŠ©‚ß‚ē‚ź‚é‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[4
Ž©‰Ę‘¢ŒŒŠ²×–EˆŚA•¹—p‘å—Ź‰»Šw—Ć–@‚ĶCLL ‚É‘Ī‚·‚鎔—Ć‚Ę‚µ‚Đ„§‚³‚ź‚Č‚¢B

„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2B
“ÆŽķ‘¢ŒŒŠ²×–EˆŚA‚ĶC—\Œć•s—Ē‚ȐõF‘ĢˆŁķ‚š‚ą‚ĀŠ³ŽŅ‚āƒvƒŠƒ“ƒAƒiƒƒOŽ”—Ć‚É’ļR«‚ĢŠ³ŽŅ‚ÅŠ®‘S‘tŒų/ •”•Ŗ‘tŒųiCR/PRj‚É’B‚µ‚½ź‡‚ĶC’·Šś—\Œć‚š‰ü‘P‚·‚鎔—Ć–@‚Ę‚µ‚čl—¶‚³‚ź‚éB

y‰š ąz

CLL ‚É‘Ī‚·‚鎩‰Ę‘¢ŒŒŠ²×–EˆŚA•¹—p‘å—Ź‰»Šw—Ć–@ihigh-dose chemotherapy with autologous hematopoietic stem cell transplantationFHDC/AHSCTj‚Ģ—LŒų«‚ĶCƒ‰ƒ“ƒ_ƒ€‰»”äŠrŽŽŒ±‚ŏ‰”­1j2j‚Ø‚ę‚ŃÄ”­1jŠ³ŽŅ‚ÅŒŸŲ‚³‚źC–³ƒCƒxƒ“ƒg¶‘¶ŠśŠŌiEFSj‚ā–³‘ˆ«¶‘¶ŠśŠŌiPFSj‚ĶHDC/AHSCT ŒQ‚Ŗ—LˆÓ‚É—D‚ź‚Ä‚¢‚½‚ŖC‘S¶‘¶ŠśŠŌiOSj‚Ģ‰ü‘P‚Ķ‚Ż‚ē‚ź‚Č‚©‚Į‚½B–¢Ž”—Ć‚ ‚é‚¢‚ĶÄ”­‚ĢCLL Š³ŽŅ‚É‘Ī‚·‚é’nŒÅ‚ß—Ć–@‚Ę‚µ‚ÄHDC/AHSCT ‚šs‚Į‚Ä‚ąC‘S¶‘¶ŠśŠŌ‚š‰„’·‚·‚éŒų‰Ź‚Ķ”F‚ß‚ē‚ź‚øCœ‘ˆŁŒ`¬ĒŒóŒQ‚š‚Ķ‚¶‚ß‚Ę‚·‚é“ńŽŸ”­‚Ŗ‚ń‚Ģ•p“x‚Ŗ‚‚Ü‚éŠėŒÆ‚Ŗ‚ ‚邽‚߁CCLL ‚É‘Ī‚·‚鎔—Ć‚Ę‚µ‚ÄHDC/AHSCT ‚Ķ„§‚³‚ź‚Č‚¢B

Œć•ūŽ‹“I‰šĶ3j‚Ę‘O•ūŽ‹“I‰šĶ4j‚ĢŒ‹‰Ź‚©‚ē“ÆŽķ‘¢ŒŒŠ²×–EˆŚAiallogeneic hematopoietic stem cell transplantationFallo-HSCTj‚šs‚¤‚ĘˆŚA•Š‘Ī”’ŒŒ•aiGVLjŒų‰Ź‚Ŗ“¾‚ē‚źCCLL Š³ŽŅ‚Ģ’·Šś¶‘¶‚ŖŠś‘Ņ‚³‚ź‚éB‚Ü‚½‘½•Ļ—Ź‰šĶ‚Å‚ĶCõF‘Ģ17p Œ‡Žø‚Ģ—L–³‚Ø‚ę‚ŃƒvƒŠƒ“ƒAƒiƒƒOŽ”—Ć‚É‘Ī‚·‚é’ļR«‚Ķ—\Œć•s—ĒˆöŽq‚Ę‚µ‚ÄŒŸo‚³‚ź‚øCallo-HSCT ‚šs‚¤‚±‚Ę‚Å‚±‚ź‚ē‚Ģ—\Œć•s—ĒˆöŽq‚ĶŽ•ž‚³‚ź‚é‚ʍl‚¦‚ē‚ź‚éBõF‘Ģ17p Œ‡Žø‚šŽ‚Āź‡‚Ķ‘Šś‚ɉ»Šw—Ć–@’ļR«‚Ę‚Č‚é‚½‚߁C‰‰ńŽ”—Ć‚É‚ę‚芮‘S‘tŒų/ •”•Ŗ‘tŒųiCR/PRj‚É’B‚µ‚½ź‡Callo-HSCT ‚šs‚¤‚±‚Ę‚šl—¶‚·‚邱‚Ę‚Ŗ–]‚Ü‚µ‚¢B‚Ü‚½CƒvƒŠƒ“ƒAƒiƒƒOŽ”—Ć‚É’ļR«‚ą‚µ‚­‚ĶŽ”—ĆŒć1 ”NˆČ“ą‚ɍĔ­‚·‚銳ŽŅ‚ĶC“ńŽŸŽ”—Ć‚ÅCR/PR ‚Ŗ“¾‚ē‚ź‚½ź‡‚Éallo-HSCT ‚šs‚¤‚±‚Ę‚Ŗ„§‚³‚ź‚éB

”ńŠ°‰šŠś‚ĢŠ³ŽŅ‚Ø‚ę‚ŃŒa5 cm ‚š’“‚¦‚郊ƒ“ƒpßŽī’Æ‚Ŗ‚ ‚銳ŽŅ‚ĶCallo-HSCT ‚šs‚Į‚Ä‚ąÄ”­‚·‚éŠėŒÆ«‚Ŗ‚‚­’·Šś—\Œć‚Ķ•s—Ē‚Å‚ ‚é5jB

œ‘”j‰ó“I‘¢ŒŒŠ²×–EˆŚA‚Å‚ĶÄ”­ˆČŠO‚ĢŒ“ˆö‚Å‚ĢŽ€–S—¦‚Ŗ‚‚¢‚½‚߁C‘唼‚Ģ—Տ°ŽŽŒ±‚Å‚Ķœ‘”ń”j‰ó“I‘¢ŒŒŠ²×–EˆŚA‚ŖŽĄŽ{‚³‚ź‚Ä‚¢‚é‚ŖC—¼ŽŅ‚Ģ”äŠrŽŽŒ±‚Ķs‚ķ‚ź‚Ä‚¢‚Č‚¢‚½‚߁C‚Ē‚æ‚ē‚Ŗ‚ę‚¢‚©‚Ķ•s–¾‚Å‚ ‚éB

yŽQl•¶Œ£z

1j Michallet M, et al ; EBMT Chronic Leukemia Working Party. Autologous hematopoietic stem cell transplantation in chronic lymphocytic leukemia : results of European intergroup randomized trial comparing autografting versus observation. Blood. 2011 ; 117i5j : 1516-21.i1iiDij

2j Sutton L, et al ; Société Française de Greffe de Moelle et de Thérapie CellulaireiSFGM-TCj and Groupe Français dfétude de la Leucémie Lymphoïde Chronique iGFLLCj. Autologous stem cell transplantation as a first-line treatment strategy for chronic lymphocytic leukemia : a multicenter, randomized, controlled trial from the SFGM-TC and GFLLC. Blood. 2011 ; 117i23j : 6109-19.i1iiDij

3j Schetelig J, et al. Allogeneic hematopoietic stem-cell transplantation for chronic lymphocytic leukemia with 17p deletion : a retrospective European Group for Blood and Marrow Transplantation analysis. J Clin Oncol. 2008 ; 26i31j : 5094-100.i3iiiAj

4j Dreger P, et al ; German CLL Study Group. Allogeneic stem cell transplantation provides durable disease control in poor-risk chronic lymphocytic leukemia : long-term clinical and MRD results of the German CLL Study Group CLL3X trial. Blood. 2010 ; 116i14j : 2438-47.i3iiiAj

5j Sorror ML, et al. Five-year follow-up of patients with advanced chronic lymphocytic leukemia treated with allogeneic hematopoietic cell transplantation after nonmyeloablative conditioning. J Clin Oncol. 2008 ; 26i30j : 4912-20.i3iiiAj


6
  œ‘ˆŁŒ`¬ĒŒóŒQ
imyelodysplastic syndromesFMDSj

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œ‘ˆŁŒ`¬ĒŒóŒQiMyelodysplastic syndromesFMDSj‚Ķ‘¢ŒŒ×–E‚ĢˆŁķ‚Č‘B‚ĘƒAƒ|ƒg[ƒVƒX‚É‚ę‚Į‚Ä“Į’„•t‚Æ‚ē‚ź‚é’PƒNƒ[ƒ“«‚ĢŽ¾Š³‚ŁC–¢n‚Č‘¢ŒŒ×–E‚ɐ¶‚¶‚½ˆŁķ‚ŖŒ“ˆö‚Å‚ ‚é‚ʍl‚¦‚ē‚ź‚Ä‚¢‚é1jB1982 ”N‚ĢFrench-American-British iFABj•Ŗ—Ž2j‚É‚ę‚Į‚ÄŽ¾Š³ŠT”O‚Ŗ–¾‚ē‚©‚Ę‚Č‚čCŒ»Ż‚ĶWHO •Ŗ—Ži2001j3j‚ĘWHO •Ŗ—Ži2008j4j‚š—p‚¢‚ÄŽę‚čˆµ‚ķ‚ź‚Ä‚¢‚éB‚µ‚©‚µCFAB •Ŗ—Ž‚É‚ę‚éŽę‚čˆµ‚¢‚ą•¹—p‚³‚ź‚Ä‚¢‚é‚Ģ‚ŖŒ»ó‚Å‚ ‚éB’Pˆź‚ ‚é‚¢‚Ķ•””Œn“‚ĢŒŒ‹…Œø­CŒ`‘ŌŠw“IˆŁŒ`¬Cœ‘‚É‚Ø‚Æ‚é–³Œų‘¢ŒŒC‹}«”’ŒŒ•a“]‰»‚ĢƒŠƒXƒN‚š“Į’„‚Ę‚µ‚Ä‚¢‚é‚ŖC’PˆźŽ¾Š³‚Å‚Ķ‚Č‚­•””‚ĢŽ¾Š³‚©‚ē‚Č‚éĒŒóŒQ‚ĢW‚Ü‚čiSyndromesj‚Ę‘Ø‚¦‚ē‚ź‚Ä‚¢‚éB‚µ‚½‚Ŗ‚Į‚āCŒ»Ż‚Ģ•aŒ^•Ŗ—Ž‚Ģ‚Ż‚Å‚Ķ—Տ°“I‚Č‘Ī‰ž‚šŒˆ’č‚·‚é‚É‚Ķ\•Ŗ‚Å‚Ķ‚Č‚¢‚ʍl‚¦‚ē‚ź‚éBMDS ‚Ģ”¼”ˆČć‚ɐõF‘ĢˆŁķ‚Ŗ‚ ‚čC–¢•Ŗ‰»‚Č‘¢ŒŒ×–E‚ɐ¶‚¶‚½ˆā“`ŽqˆŁķ‚Ŗ”­Ē‚ÉŠÖ—^‚·‚é‚ʍl‚¦‚ē‚ź‚Ä‚¢‚éB‚³‚ē‚ɁCõF‘ĢŒŸøƒŒƒxƒ‹‚Å‚ĶŠm”F‚Å‚«‚Č‚¢V‚½‚Čˆā“`ŽqˆŁķ‚ŖŽŸX‚É“Æ’č‚³‚ź‚Ä‚Ķ‚¢‚é‚ŖC‚»‚Ģ‘S–e‚Ķ–¢‚¾–¾‚ē‚©‚É‚Č‚Į‚Ä‚¢‚Č‚¢B

ˆź”Ź‚ɐf’f‚ĶWHO •Ŗ—Ži2008j‚ÉŠī‚Ć‚«CŒŒ‹…Œø­C––½ŒŒ‚ʍœ‘‚Ģ‰č‹…Š„‡C‘¢ŒŒ×–E‚ĢˆŁŒ`¬CõF‘ĢˆŁķ‚É‚ę‚Į‚Ä‚Č‚³‚źCˆź•”‚Å‚ĶFAB •Ŗ—Ž‚É‚ę‚éf’f‚ąŽQl‚Ę‚³‚ź‚Ä‚¢‚éBMDS ‚ĶŽķX‚ĢŒŒ‰tŽ¾Š³‚Ę‹«ŠE‚šŚ‚µ‚Ä‚Ø‚čCŒo‰ßŠĻŽ@‚ā‘¼Ž¾Š³‚ĢœŠO‚Ę‚Ę‚ą‚ɁCŒ»Ż‚ąf’f‚Ģd—v‚Č•”•Ŗ‚ĶŒ`‘ŌŠw“I‚Č”»’f‚É•‰‚¤‚Ę‚±‚ė‚Ŗ‘å‚«‚¢1jBŠm’čf’f‚Ŗ“¾‚ē‚ź‚½Œć‚ĶCf’f‚É—p‚¢‚ē‚ź‚½ŒŒ‰tŠŒ©Cœ‘ŠŒ©CõF‘ĢˆŁķ‚Č‚Ē‚É‚ę‚Į‚Ä—\Œć—\‘Ŗ‚Ŗs‚ķ‚źCŽ”—Ć•ūj‚ŖŒˆ’č‚³‚ź‚Ä‚¢‚­B—\Œć‚ĶCŒŒ‹…Œø­‚ÉŠÖ˜A‚µ‚½Ž–ŪiŠ“õĒCoŒŒ‚Č‚Ēj‚Ę”’ŒŒ•a‰»‚É‚ę‚Į‚Ä‘å‚«‚­Œˆ’č‚³‚ź‚é‚ŖC–{Ž¾Š³‚Ķ‚—īŽŅ‚É‘½‚¢‚±‚Ę‚ę‚čC‡•¹Ē‚Č‚ĒŠ³ŽŅ”wŒi‚ą—\Œć‚É‘å‚«‚ȉe‹æ‚šŽ‚Į‚Ä‚¢‚éB

Œ»Ż‚Å‚ąŖŽ”—Ć–@‚Ķ“ÆŽķ‘¢ŒŒŠ²×–EˆŚA‚Ģ‚Ż‚Å‚ ‚é‚ŖCŠ³ŽŅW’c‚Ģ”N—ī‚Č‚Ē‚©‚ē“ÆŽķˆŚA‚Ģ‰¶Œb‚É‚ ‚ø‚©‚éĒ—į‚Ķˆź•”‚ÉŒĄ‚ē‚ź‚Ä‚¢‚éBˆź•ūCÅ‹ßCMDS ‚É‘Ī‚·‚éV–ņ‚ŖŠJ”­‚³‚źŽ”—Ć‚É‚ąV‚½‚Č“WŠJ‚Ŗ‚Ż‚ē‚ź‚Ä‚¢‚éB

yŽQl•¶Œ£z

1j ¬ąVŒh–ē•ŅW@“Į”­«‘¢ŒŒįŠQŽ¾Š³‚Ģf—Ć‚ĢŽQĘƒKƒCƒh@•½¬22 ”N“x‰ü’ł”Å

2j Bennett JM, et al. Proposals for the classification of the myelodysplastic syndromes. Br J Haematol. 1982 ; 51i2j : 189-99.

3j Jaff e WS, et al, eds. World Health Organization classification of Tumours. Pathology and genetics, Tumor of Haematopoietic and lymphoid Tissues. Lyon, IARC ; 2001.

4j Swerdlow SH, et al. eds. WHO classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon, IARC ; 2008.



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i¦jCQ”Ō†iƒsƒ“ƒNF•”•Ŗj‚šƒNƒŠƒbƒN‚·‚é‚ʁC‰šą‰ę–Ź‚ÖˆŚ“®‚µ‚Ü‚·

œ‘ˆŁŒ`¬ĒŒóŒQ‚ĢƒAƒ‹ƒSƒŠƒYƒ€ CQ1 CQ3 CQ4 CQ5 CQ6 CQ7 CQ8 CQ9

MDS ‚Ķ‘½—l‚Č•a‘Ō‚š—L‚·‚鎾Š³‚ĢW‡‘Ģ‚Å‚ ‚čCŽ”—Ć•ūj‚šŒˆ’č‚·‚éć‚Å‚Ķf’f‚Ę•aŒ^•Ŗ—Ž‚Ģ‚Ż‚Å‚Ķ•s\•Ŗ‚Å‚ ‚éB‚»‚Ģ‚½‚߁CŽå‚ɗՏ°“I‚ČˆöŽq‚š—p‚¢‚Ä—\Œć—\‘Ŗ‚Ŗ‚Č‚³‚ź‚éiCQ1jB•””‚Ģ—\Œć—\‘ŖƒXƒRƒAƒŠƒ“ƒOƒVƒXƒeƒ€‚Ŗ’ń„‚³‚ź‚Ä‚Ø‚čC‚»‚ź‚¼‚ź‚É“Į’„‚Ŗ‚Ż‚ē‚ź‚é‚ŖC—Տ°“I‚Č‘Ī‰ž‚Ķ’įƒŠƒXƒN‚ʍ‚ƒŠƒXƒN‚É•Ŗ‚Ƃčl—¶‚³‚ź‚邱‚Ę‚Ŗ‘½‚¢B•p—p‚³‚ź‚é‚Ģ‚ĶInternational prognositic scoring systemiIPSSj‚É‚Ø‚Æ‚éLow, IntermediateiIntj-1 ‚š’įƒŠƒXƒNCInt-2, High ‚š‚ƒŠƒXƒN‚Ę‚·‚é‚ą‚Ģ‚Å‚ ‚éiCQ1jB

’įƒŠƒXƒNĒ—į‚É‚Ø‚¢‚Ä‚ĶŒŒ‹…Œø­‚É‘Ī‚·‚é‘Ī‰žC‚»‚Ģ‰ü‘P‚šŽ”—Ć‚Ģ‘ęˆź–Ś•W‚Ę‚µC‚ƒŠƒXƒN—į‚Å‚Ķ”’ŒŒ•a“]‰»ƒŠƒXƒN‚Ŗ‚‚¢‚±‚Ę‚ę‚čC‚ę‚čĻ‹É“I‚ČŽ”—Ć•ūj‚Ŗ‚Ę‚ē‚ź‚éB

ŒŒ‹…Œø­‚É‘Ī‚µ‚Ä‚ĢŠī–{“I‚ČŽxŽ—Ć–@‚Ķ—AŒŒ‚Å‚ ‚é‚ŖCŌŒŒ‹…—AŒŒ‚É”ŗ‚¤—AŒŒŒć“S‰ßčĒ‚ĶMDS ‚Ģ—\Œć‚ÉŠÖ˜A‚µ‚Ä‚¢‚é‰Ā”\«‚Ŗ‚ ‚éB‚»‚Ģ‚½‚߁CMDS ‚Ģ—AŒŒŒć“S‰ßčĒ‚É‘Ī‚µ‚Ä‚Ķ“KŲ‚Č“SƒLƒŒ[ƒg—Ć–@‚ŖŽ”—Ć‚Ģ‘I‘šŽˆ‚Ę‚µ‚čl‚¦‚ē‚ź‚éiCQ2jB’įƒŠƒXƒN—į‚ĢŒŒ‹…Œø­‚É‘Ī‚µ‚Ä‚Ķ–ʉu—}§—Ć–@iCQ3C‘“ą“K‰žŠOjCƒTƒCƒgƒJƒCƒ“—Ć–@iCQ4C‘“ą“K‰žŠOjC‚Ü‚½C5 ”ŌõF‘Ģ’·˜r‚ĢŒ‡‘¹‚š”ŗ‚¤5q- ĒŒóŒQ‚Å‚Ż‚ē‚ź‚錌‹…Œø­‚Ö‚ĢƒŒƒiƒŠƒhƒ~ƒhiLENjiCQ5jCˆź•”‚Ģ’įƒŠƒXƒN—į‚É‘Ī‚·‚éƒAƒUƒVƒ`ƒWƒ“iAZAj“Š—^iCQ6j‚Č‚Ē‚Ŗs‚ķ‚ź‚éB‚»‚ź‚¼‚ź‚ĢŽ”—Ć‚É‚ę‚Į‚Ĉź’č‚ĢŒŒ‹…‰ń•œ‚Ŗ‚Ż‚ē‚ź‚é‚ŖC‚±‚ĢŒQ‚É‚Ā‚¢‚Ä‚Ķ‚±‚¤‚µ‚½Ž”—Ć‚É‚ę‚Į‚摶ŠśŠŌ‚Ģ‰„’·‚Ŗ‚Ż‚ē‚ź‚é‚Ģ‚©C–¾‚ē‚©‚ČƒGƒrƒfƒ“ƒX‚Ķ‚Č‚¢B

‚ƒŠƒXƒNĒ—į‚Ķ—\Œć‚Ŗˆ«‚¢‚½‚߁CĻ‹É“I‚Č‘Ī‰ž‚Ŗ‚Č‚³‚ź‚éB”N—ī‚āŠ³ŽŅ”wŒiCƒhƒi[‚Č‚Ē‚ĢšŒ‚Ŗ‹–‚¹‚Ī“ÆŽķ‘¢ŒŒŠ²×–EˆŚAiCQ7j‚ĢĻ‹É“I‚Č“K‰ž‚Ŗl—¶‚³‚ź‚éB‚±‚ź‚Ü‚Å‚Ģ‚Ę‚±‚ėŽ”–ü‚Ŗ“¾‚ē‚ź‚鎔—Ć–@‚Ķ“ÆŽķ‘¢ŒŒŠ²×–EˆŚA‚Ģ‚Ż‚Å‚ ‚é‚ŖCˆŚA‚Ŗ‚Č‚³‚ź‚Č‚¢—į‚É‘Ī‚µ‚Ä‚ĶAZA ‚Ŗ‘I‘š‚³‚ź‚éiCQ8jBAZA ‚Ķ‘O•ūŽ‹“IŽŽŒ±‚É‚ę‚Į‚č‚ƒŠƒXƒNĒ—į‚Ģ—\Œć‚š‰ü‘P‚·‚邱‚Ę‚ŖŽ¦‚³‚ź‚Ä‚¢‚éB5 ”ŌõF‘Ģ’·˜r‚ĢŒ‡‘¹‚š”ŗ‚Į‚½—į‚Å‚ĶLEN ‚ą“Š—^‰Ā”\‚Å‚ ‚éiCQ9jBˆź•”‚Ģ‚ƒŠƒXƒN—į‚É‘Ī‚µ‚Ä‚Ķ”’ŒŒ•aŽ”—Ć‚É€‚¶‚½RŽīį‡–ņ“Š—^‚ąl—¶‚³‚ź‚éiCQ10jB


CQ1

MDS ‚Ģ—\Œć—\‘Ŗ–@CƒŠƒXƒN•Ŗ—Ž‚Ę‚µ‚ÄŠ©‚ß‚ē‚ź‚é‚Ģ‚Ķ‰½‚©


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‘Ū—\Œć—\‘ŖƒXƒRƒAƒŠƒ“ƒOƒVƒXƒeƒ€iInternational prognostic scoring systemFIPSSj‚āWHO •Ŗ—Ž‚ÉŠī‚Ć‚­—\Œć—\‘ŖƒXƒRƒAƒŠƒ“ƒOƒVƒXƒeƒ€iWHO classification-based prognostic scoring systemFWPSSj‚Ģ‚ꂤ‚É•””‚ĢˆöŽq‚š‘g‚Ż‡‚ķ‚¹‚Ä—\Œć‚š—\‘Ŗ‚·‚éƒXƒRƒAƒŠƒ“ƒOƒVƒXƒeƒ€‚ŖŠ©‚ß‚ē‚ź‚éB“Į‚ɁCIPSS ‚Ķ•p—p‚³‚ź‚Ä‚¢‚éB

y‰š ąz

MDS ‚Ķ‘½—l‚ČŽ¾Š³’PˆŹ‚ĢW‡‘Ģ‚Å‚ ‚čC’P‚Č‚é•aŒ^•Ŗ—Ž‚Å‚Ķ\•Ŗ‚Č—\Œć—\‘Ŗ‚Ķ‚Å‚«‚Č‚¢‚ʍl‚¦‚ē‚ź‚Ä‚¢‚éB‘¢ŒŒ×–E‚ĢŒ`‘Ō“I‚Č“Į’„C“Į‚ɉ苅Š„‡CŒŒ‹…Œø­‚Ģ’ö“x‚āŒø­‚µ‚Ä‚¢‚éŒn“”CõF‘ĢŠŒ©‚Ķ—\Œć‚ĘŠÖ˜A‚·‚éB‚»‚±‚ŁC‚»‚ź‚ē‚šƒXƒRƒA‰»‚µC‚»‚Ģ‡Œv“_”‚ÅMDS ‚Ģ—\Œć‚š—\‘Ŗ‚·‚é•ū–@‚ŖŠJ”­‚³‚ź‚½BIPSS ‚ĘŒÄ‚Ī‚ź‚é‚ą‚Ģ‚ŁCMDS ‚šœ‘‰č‹…Š„‡CŒŒ‹…Œø­‚ĢŒn“”CõF‘ĢƒOƒ‹[ƒv‚Ę‚¢‚¤3 ˆöŽq‚Ģ“_”‚É‚ę‚Į‚Ä4 ŒQ‚É‘w•Ź‰»‚·‚é‚ą‚Ģ‚Å‚ ‚é1jBFAB •Ŗ—Ž‚ÉŠī‚Ć‚¢‚čģ¬‚³‚ź‚Ä‚¢‚邽‚߁CŒ»Ż‚ĢWHO •Ŗ—Ži2008j‚Å‚Ķ‹}«œ‘«”’ŒŒ•a‚ÉŠÜ‚Ü‚ź‚é‰č‹…20`30“‚Ģ—į‚ąŽę‚čˆµ‚Į‚Ä‚¢‚éBIPSS ‚Ķ‚±‚ź‚Ü‚Å‘½”‚Ģ—Տ°Œ¤‹†‚Å—p‚¢‚ē‚ź‚Ä‚¢‚éB

—\Œć—\‘ŖˆöŽq‚ÉWHO •Ŗ—Ži2001j‚Ę—AŒŒˆĖ‘¶«‚šĢ—p‚µ‚½‚Ģ‚ŖWPSS ‚ŁC‰fŽž‚Ī‚©‚č‚Å‚Č‚­•aŠśi“WŽž‚É‚ą“K‰ž‚Å‚«‚é‚Ę‚¢‚¤“Į’„‚Ŗ‚ ‚é2jB‚µ‚©‚µCŒ»Ż‚ĶWHO •Ŗ—Ži2008j‚ŖŠł‚ɏo‚Ä‚Ø‚čCWPSS ‚Ķ\•Ŗ‚Č—˜—p‚Ŗ‚Č‚³‚ź‚Ä‚¢‚Č‚¢B

yŽQl•¶Œ£z

1j Greenberg P, et al. International scoring system for evaluating prognosis in myelodysplastic syndromes. Blood. 1997 ; 89i6j : 2079-88.i3iiAj

2j Malcovati L, et al. Time-dependent prognostic scoring system for predicting survival and leukemic evolution in myelodysplastic syndromes. J Clin Oncol. 2007 ; 25i23j : 3503-10.i3iiAj


CQ2

—AŒŒ‚É‚ę‚é“S‰ßčĒ‚Ö‚Ģ“SƒLƒŒ[ƒgÜ‚Ŗ“K‰ž‚Ę‚³‚ź‚éó‘Ō‚Ķ‰½‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2B
ˆź”NˆČć‚Č‚Ēˆź’č‚Ģ—\Œć‚ŖŠś‘Ņ‚³‚ź‚©‚ĀC’芜“I‚ȐŌŒŒ‹…—AŒŒ‚š•K—v‚Ę‚·‚éMDS ‚ŁC—AŒŒ‚É‚ę‚é“S‰ßčó‘Ō‚ÉŽŠ‚Į‚½ź‡‚É‚Ķ“SƒLƒŒ[ƒg—Ć–@‚šŽĄŽ{‚·‚éB

y‰š ąz

ˆź’č—Ź‚ĢŌŒŒ‹…—AŒŒ‚šŽó‚Æ‚½MDS Š³ŽŅ‚Ķ“S‰ßčó‘Ō‚ɊׂĮ‚Ä‚Ø‚čCMDS Š³ŽŅ‚Å‘ŸŠķ•s‘S‚š¶‚¶‚½—į‚āŽ€–S—į‚Å‚Ķ“S‰ßčó‘Ō‚ɊׂĮ‚Ä‚¢‚鏼‡‚Ŗ‚‚¢1jB‘gD‚Ģ‰ßč“S‚ĶŠˆ«Ž_‘fŽķ‚ĢŽY¶‚š˜“i‚³‚¹‚邱‚Ę‚Å‘gDįŠQ‚šˆų‚«‹N‚±‚µ‚Ä‚¢‚é‚ʍl‚¦‚ē‚ź‚Ä‚¢‚éB“SƒLƒŒ[ƒgÜ‚š“Š—^‚·‚邱‚Ę‚Å‘Ģ“ą‚Ģ“S‰ßč‚Ŗ‰ü‘P‚³‚ź2j3jC¶‘¶ŠśŠŌ‚Ģ‰„’·‚ÉŠń—^‚·‚é‰Ā”\«‚ŖŽw“E‚³‚ź‚Ä‚¢‚é4jB

“S‰ßčĒ‚Ģf’f‹K€‚ā“SƒLƒŒ[ƒg—Ć–@‚ĢŠJŽn‹K€‚Ę“K‰žCŽĄŪ‚ĢŽ”—Ć‚ÉŠÖ‚µ‚Ä‚ĶCu—AŒŒŒć“S‰ßčĒ‚Ģf—ĆƒKƒCƒhv‚Ę‚µ‚Ä–{–M‚Ģ“Į”­«‘¢ŒŒįŠQ‚ÉŠÖ‚·‚é’²øŒ¤‹†”Ē‚ę‚čf—ĆƒKƒCƒh‚Ŗo‚³‚ź‚Ä‚¢‚é5jB‚»‚ź‚É‚ę‚é‚ʁCŌŒŒ‹…—AŒŒˆĖ‘¶‚Ę‚Č‚Į‚½Š³ŽŅiŒŽ2 ’PˆŹˆČć‚Ģ—AŒŒ‚š6 ƒJŒŽˆČćŒp‘±j‚Ģ‚¤‚æ1 ”NˆČć‚Ģ—]–½‚ŖŠś‘Ņ‚Å‚«‚é—į‚É‚Ø‚¢‚āC‘ŌŒŒ‹…—AŒŒ—Ź‚Ŗ40 ’PˆŹ‚š’“‚¦ŒŒ“ƒtƒFƒŠƒ`ƒ“’l‚Ŗ2 ƒJŒŽˆČć‚É‚ķ‚½‚Į‚Ä1,000 ng/mL ‚š’“‚¦‚鏼‡‚É“SƒLƒŒ[ƒg—Ć–@‚ĢŠJŽn‚Ŗ„§‚³‚ź‚Ä‚¢‚éBŽ”—ĆŒų‰Ź‚ĶŒŒ“ƒtƒFƒŠƒ`ƒ“’l‚Å‚Č‚³‚źC500`1,000 ng/mL ‚ĢˆŪŽ‚Ŗ–Ś•W‚Ę‚³‚ź‚Ä‚¢‚éB

‚µ‚©‚µCMDS ‚É‚Ø‚¢‚Ä—AŒŒŒć“S‰ßčĒ‚É‘Ī‚·‚é“SƒLƒŒ[ƒg—Ć–@‚Ŗ¶‘¶‰„’·‚ÉŠń—^‚·‚é‚©‚Ē‚¤‚©‚ĶC‘O•ūŽ‹“I‚Čƒ‰ƒ“ƒ_ƒ€‰»ŒŸŲ‚Ŗ‚Č‚³‚ź‚Ä‚¢‚Č‚¢B

yŽQl•¶Œ£z

1j Takatoku M, et al ; Japanese National Research Group on Idiopathic Bone Marrow Failure Syndromes. Retrospective nationwide survey of Japanese patients with transfusion-dependent MDS and aplastic anemia highlights the negative impact of iron overload on morbidity/mortality. Eur J Haematol. 2007 ; 78i6j : 487-94.i3iiiDivj

2j Gattermann N, et al ; EPIC study investigators. Deferasirox in iron-overloaded patients with transfusiondependent myelodysplastic syndromes : Results from the large 1-year EPIC study. Leuk Res. 2010 ; 34i9j : 1143-50.i3iiDivj

3j Greenberg PL, et al. Prospective assessment of effects on iron-overload parameters of deferasirox therapy in patients with myelodysplastic syndromes. Leuk Res. 2010 ; 34i12j : 1560-5.i3iiDivj

4j Rose C, et al ; GFM iGroupe Francophone des Myélodysplasiesj. Does iron chelation therapy improve survival in regularly transfused lower risk MDS patients? A multicenter study by the GFM iGroupe Francophone des Myélodysplasiesj. Leuk Res. 2010 ; 34i7j : 864-70.i3iiAj

5j Œ¤‹†‘ć•\ŽŅ@¬ąVŒh–ē@—AŒŒŒć“S‰ßčĒ‚Ģf—ĆƒKƒCƒh@Œś¶˜J“­‰ČŠwŒ¤‹†”ļ•ā•‹ą“ļŽ”«Ž¾Š³Ž•žŒ¤‹†Ž–‹Ę“Į”­«‘¢ŒŒįŠQ‚ÉŠÖ‚·‚é’²øŒ¤‹†”Ē@•½¬20 ”N“xiƒKƒCƒhƒ‰ƒCƒ“j


CQ3

’įƒŠƒXƒNMDS ‚ĢŽ”—Ć‚É‚Ø‚¢‚Ėʉu—}§—Ć–@‚ĶŠ©‚ß‚ē‚ź‚é‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2A
MDS ‚Ģˆź•”‚ĢĒ—į‚É‘Ī‚µ‚Ä‚Ķ‘¢ŒŒ‚Ģ‰ń•œ‚ɖʉu—}§—Ć–@‚ŖŠ©‚ß‚ē‚ź‚éB“Į‚ÉHLA-DR15CŌŒŒ‹…—AŒŒ—š‚Ģ’Z‚¢—įCŽį”N—į‚Å‚ĢŒų‰Ź‚ŖŠś‘Ņ‚³‚ź‚éi‘“ą“K‰žŠOjB

y‰š ąz

MDSC“Į‚ɉ苅‘‰Į‚Ģ‚Ż‚ē‚ź‚Č‚¢’įƒŠƒXƒN—į‚Ģˆź•”‚É‚Ø‚¢‚āCR‹¹‘B×–EƒOƒƒuƒŠƒ“iantithymocyte globurinFATGj‚É‚ę‚Į‚Ä‘¢ŒŒ‰ń•œ‚Ŗ‚Ż‚ē‚ź‚é1jB‚Ü‚½CƒVƒNƒƒXƒ|ƒŠƒ“‚š—p‚¢‚½–ʉu—}§—Ć–@‚ąˆź’č‚Ģ‘¢ŒŒ‰ń•œŒų‰Ź‚šŽ¦‚·2jB—¼ŽŅ‚Ģ‘g‚Ż‡‚ķ‚¹‚É‚ę‚郉ƒ“ƒ_ƒ€‰»”äŠrŽŽŒ±‚Å‚ą—LˆÓ‚Č‘¢ŒŒ‰ń•œ‚Ŗ“¾‚ē‚ź‚½‚ŖC‘S¶‘¶Š„‡iOSjC–³—AŒŒ¶‘¶Š„‡‚Ķ‰ü‘P‚³‚ź‚Č‚©‚Į‚½3jB

‘¢ŒŒ‰ń•œ‚ĶC‘½•Ļ—Ź‰šĶ‚É‚ę‚é‚ĘHLA-DR15CŽį”N—įCŌŒŒ‹…—AŒŒ—š‚Ģ’Z‚¢—į‚Å‚ę‚čŒų‰Ź‚Ŗ“¾‚ē‚ź‚Ä‚¢‚½4jB‘“ą‚Ģ‰šĶ‚Å‚ĶHLA-DRB1– 15F01 ‚Ŗ‘¢ŒŒ‰ń•œ‚ĘŠÖ˜A‚µ‚Ä‚¢‚½2jB‚»‚Ģ‘¼CPNH iparoxysmal nocturnal hemoglobinuriajŒ^ŌŒŒ‹…‚Ģ‘¶Ż‚ŖŽ”—Ć”½‰ž«‚ĘŠÖ˜A‚·‚é5jB

yŽQl•¶Œ£z

1j Molldrem JJ, et al. Antithymocyte globulin for patients with myelodysplastic syndrome. Br J Haematol. 1997 ; 99i3j : 699-705.i3iiDivj

2j Shimamoto T, et al. Cyclosporin A therapy for patients with myelodysplastic syndrome : multicenter pilot studies in Japan. Leuk Res. 2003 ; 27i9j : 783-8.i3iiDivj

3j Passweg JR, et al. Immunosuppressive therapy for patients with myelodysplastic syndrome : a prospective randomized multicenter phase Ⅲ trial comparing antithymocyte globulin plus cyclosporine with best supportive care--SAKK 33/99. J Clin Oncol. 2011 ; 29i3j : 303-9.i1iiDivj

4j Saunthararajah Y, et al. HLA-DR15 iDR2j is overrepresented in myelodysplastic syndrome and aplastic anemia and predicts a response to immunosuppression in myelodysplastic syndrome. Blood. 2002 ; 100i5j : 1570-4.i3iiiDivj

5j Wang H, et al. Clinical significance of a minor population of paroxysmal nocturnal hemoglobinuria-type cells in bone marrow failure syndrome. Blood. 2002 ; 100i12j : 3897-902.i3iiiDivj


CQ4

’įƒŠƒXƒNMDS ‚Ģ•nŒŒ‚É‘Ī‚µ‚ăTƒCƒgƒJƒCƒ“—Ć–@‚ĶŠ©‚ß‚ē‚ź‚é‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2A
ŒŒ“ƒGƒŠƒXƒƒ|ƒGƒ`ƒ“”Z“x’į’li200 ‚Ü‚½‚Ķ500 mU/mL –¢–žjCŠĀó“S‰č‹…15“–¢–žCŌŒŒ‹…—AŒŒˆĖ‘¶‚Ģ‚Č‚¢‚Ü‚½‚ĶŒŽ2 ’PˆŹ’ö“x‚Ģ—AŒŒ‚š•K—v‚Ę‚·‚é•nŒŒ‚š—L‚·‚éMDS ‚É‘Ī‚µ‚Ä‚ĶƒGƒŠƒXƒƒ|ƒGƒ`ƒ“i40,000`60,000U T1`3 ‰ń“Š—^j‚Ģ“Š—^‚Ŗ•nŒŒ‚š‰ü‘P‚³‚¹‚éi‘“ą“K‰žŠOjBč÷—±‹…ƒRƒƒj[ŽhŒƒˆöŽqiG-CSFj‚Ģ•¹—p‚Ķ”½‰ž«‚šćø‚³‚¹‚éBƒ_ƒ‹ƒxƒ|ƒGƒ`ƒ““Š—^‚ą“Æ—l‚ĢŒų‰Ź‚šŽ¦‚·i‘“ą“K‰žŠOjB

y‰š ąz

MDS ‚Ģ•nŒŒ‚É‘Ī‚·‚éƒGƒŠƒXƒƒ|ƒGƒ`ƒ“iEPOj“Š—^‚ĶCˆź•”‚Ģ—į‚Å•nŒŒ‚š‰ü‘P‚·‚éB17 ‚ĢŽŽŒ±‚É‚Ø‚Æ‚é205 —į‚ĢMDS ‚É‘Ī‚·‚éEPO Ž”—Ć‚Ģ“‡‰šĶ‚Å‚Ķ33 —įi16“j‚ÉŒŒ‹…Œø­‚Ģ‰ü‘P‚Ŗ“¾‚ē‚ź‚Ä‚¢‚½1jB”½‰ž«‚ÉŠÖ‚·‚éˆöŽq‚Ģ‰šĶ‚Å”ń“S‰č‹…«•nŒŒ—įCŽ”—Ć‘OŒŒ“EPO 200 mU/mL –¢–žC—AŒŒ•K—v‚Ģ‚Č‚¢•nŒŒ—į‚É‚Ø‚¢‚Ä—LŒų«‚Ŗ—LˆÓ‚ɍ‚‚©‚Į‚½B

G-CSF ‚ĘEPO ‚Ę‚Ģ•¹—p—į‚Å‚Ķ‚»‚ĢŒų‰Ź‚Ŗ39“‚ʍ‚‚­2jC•¹—p—Ć–@‚Ģ‘ęⅡ‘ŠŽŽŒ±‚Ģ’·ŠśŠĻŽ@—į‚šC‘½•Ļ—Ź‰šĶ‚š—p‚¢‚ÄŒŸ’肵‚½‚Ę‚±‚ėŽ”—Ć—į‚Å‚Ķ”ńŽ”—Ć—į‚Ę”äŠr‚µ‚Ä—LˆÓ‚É—ĒD‚Ȑ¶‘¶‚šŽ¦‚µ‚Ä‚¢‚½3jB‚µ‚©‚µC‚±‚ĢŽ”—Ć–@‚Ģ¶‘¶‚É‘Ī‚·‚éŒų‰Ź‚Ķ‘O•ūŽ‹“I‘ęⅢ‘Š”äŠrŽŽŒ±‚Å‚ĢŒŸŲ‚Ŗ‚Č‚³‚ź‚Ä‚¢‚Č‚¢B

Ž‘±«‚ĢEPO »Ü‚Å‚ ‚éƒ_ƒ‹ƒxƒ|ƒGƒ`ƒ“300ƒŹg Tˆź‰ń“Š—^‚Ü‚½‚Ķ500ƒŹg ‚Ģ3 TŠŌ‚Ø‚«“Š—^‚É‚ąMDS ‚ĢŌŒŒ‹…‘¢ŒŒ‘£iŒų‰Ź‚Ŗ‚Ż‚ē‚ź‚é4j5jBŽ”—Ć‘OŒŒ“EPO 500 mU/mL –¢–ž‚Ģ—į‚š‘ĪŪ‚Ę‚µ‚½ƒ_ƒ‹ƒxƒ|ƒGƒ`ƒ“300ƒŹg Tˆź‰ń“Š—^‚Ģ‘ę‡U‘ŠŽŽŒ±‚Å‚Ķ62 —į’†44 —įi71“j‚Å”½‰ž‚Ŗ“¾‚ē‚ź‚½B‚±‚ĢŽŽŒ±‚Å‚ąEPO ’į’l‚ʐŌŒŒ‹…—AŒŒ”ńˆĖ‘¶‚ŖŽ”—Ć”½‰ž«‚Ģ—LˆÓ‚Č—\‘ŖˆöŽq‚Å‚ ‚Į‚½B500ƒŹg ‚Ģ3 TŠŌ‚Ø‚«“Š—^ŽŽŒ±‚Å‚Ķ‰‰ń“Š—^—į‚Ģ49“‚ɐŌŒŒ‹…‘¢ŒŒ‘£iŒų‰Ź‚Ŗ‚Ż‚ē‚ź‚½B‚Ü‚½Cƒ_ƒ‹ƒxƒ|ƒGƒ`ƒ“150ƒŹg Tˆź‰ń“Š—^ŽŽŒ±‚Å‚Ķ‘S‘Ģ‚Ģ—LŒų—¦‚Ķ40.5“‚Å‚ ‚čCEPO ’į’lCŌŒŒ‹…—AŒŒŒŽ2 ’PˆŹˆČ‰ŗCœ‘‰č‹…Š„‡Cœ‘’įŒ`¬‚Ŗ”½‰ž«—\‘ŖˆöŽq‚Å‚ ‚Į‚½6jB

—¼–ņÜ‚Ę‚ą‚É—LŠQŽ–Ū‚ĶŒy”÷‚Å‚ ‚Į‚½B‚µ‚©‚µC‚¢‚ø‚ź‚Ģ–ņÜ‚ą‘“ą‚Å‚ĶMDS ‚Ģ•nŒŒ‚É‘Ī‚µ‚Ä•ŪŒÆf—Ć‚Ę‚µ‚Ä‚Ģ“K‰ž‚Ŗ‚Č‚¢B

yŽQl•¶Œ£z

1j Hellström-Lindberg E. Efficacy of erythropoietin in the myelodysplastic syndromes : a meta-analysis of 205 patients from 17 studies. Br J Haematol. 1995 ; 89i1j : 67-71.i3iiiDivj

2j Hellström-Lindberg E, et al. A validated decision model for treating the anaemia of myelodysplastic syndromes with erythropoietin{granulocyte colony-stimulating factor : signifi cant effects on quality of life. Br J Haematol. 2003 ; 120i6j : 1037-46.i3iiiCj

3j Jädersten M, et al. Erythropoietin and granulocyte-colony stimulating factor treatment associated with improved survival in myelodysplastic syndrome. J Clin Oncol. 2008 ; 26i21j : 3607-13.i3iiiAj

4j Mannone L, et al. High-dose darbepoetin alpha in the treatment of anaemia of lower risk myelodysplastic syndrome results of a phase Ⅱ study. Br J Haematol. 2006 ; 133i5j : 513-9.i3iiiDivj

5j Gabrilove J, et al. Phase 2, single-arm trial to evaluate the effectiveness of darbepoetin alfa for correcting anaemia in patients with myelodysplastic syndromes. Br J Haematol. 2008 ; 142i3j : 379-93.i3iiiDivj

6j Musto P, et al. Darbepoetin alpha for the treatment of anaemia in low-intermediate risk myelodysplastic syndromes. Br J Haematol. 2005 ; 128i2j : 204-9.i3iiiDivj


CQ5

MDS ‚ĢŽ”—Ć‚Ę‚µ‚ăŒƒiƒŠƒhƒ~ƒh‚ĶŠ©‚ß‚ē‚ź‚é‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[1
5 ”ŌõF‘Ģ’·˜r‚ĢŒ‡Žømdel (5q)n‚š”ŗ‚¤’įƒŠƒXƒN MDS‚ŐŌŒŒ‹…—AŒŒˆĖ‘¶—į‚É‘Ī‚µ‚Ä‚ĶƒŒƒiƒŠƒhƒ~ƒh‚ŖŌŒŒ‹…‘¢ŒŒ‘£iŒų‰Ź‚šŽ¦‚µCƒŒƒiƒŠƒhƒ~ƒh‚É‚ę‚鎔—Ć‚Ŗ„§‚³‚ź‚éB10 mg/“ś‚Ģ21 “śŠŌ“Š—^‚š28“śƒTƒCƒNƒ‹‚ÅŽĄŽ{‚·‚éB

„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2B
del (5q) ‚š”ŗ‚ķ‚Č‚¢ŌŒŒ‹…—AŒŒˆĖ‘¶’įƒŠƒXƒN MDS ‚É‘Ī‚µ‚Ä‚ĶCŌŒŒ‹…—AŒŒ”ńˆĖ‘¶‚Ŗ’B¬‚³‚ź‚é—į‚Ŗ‚Ż‚ē‚ź‚é‚ŖCŒ»Žž“_‚Å‚Ķ‘ęˆź‘I‘š–ņ‚Ę‚µ‚Ä‚Ķ„§‚³‚ź‚Č‚¢i‘“ą“K‰žŠOjB

y‰š ąz

ƒŒƒiƒŠƒhƒ~ƒhiLENj‚ĶƒTƒŠƒhƒ}ƒCƒhiTHALj‚Ģ—U“±‘Ģ‚ŁC–ʉu’²ß‚š‚Ķ‚¶‚ß‚Ę‚µ‚ж‘Ģ‚É‘Ī‚µ‚Ä‘½Ź‚ČŒų‰Ź‚š”­Šö‚·‚éB5 ”ŌõF‘Ģ’·˜r‚ĢŒ‡Žø‚š”ŗ‚¤MDS ‚ŐŌŒŒ‹…—AŒŒˆĖ‘¶—į‚š‘ĪŪ‚Ę‚µ‚½‘ęⅡŽŽŒ±1jC‘ęⅢ‘ŠŽŽŒ±2j‚É‚Ø‚¢‚ĐŌŒŒ‹…—AŒŒ—Ź‚ĢŒø­Œų‰Ź‚Ŗ–¾‚ē‚©‚É”F‚ß‚ē‚ź‚Ä‚¢‚éB‚Ü‚½C‘“ą‚Å‚ą­”—į‚Č‚Ŗ‚ē‘ęⅡ‘ŠŽŽŒ±‚ŖŽĄŽ{‚³‚ź‚½3jB

•Ä‘‚š’†S‚Ę‚µ‚½‘ęⅡ‘ŠŽŽŒ±‚É‚Ø‚¢‚Ä‚Ķ148 —į‚Ŗ“o˜^‚³‚źCŌŒŒ‹…Œn‰ü‘P‚Ŗ67“‚É‚Ż‚ē‚źC‚³‚ē‚É45 —į‚ōזEˆā“`Šw“IŠ°‰šiCyRj‚Ŗ“¾‚ē‚ź‚½1jB

LEN 10 mg/“śC5 mg/“śCƒvƒ‰ƒZƒ{‚É‚ę‚é“ńd–ÓŒŸŽŽŒ±‚Å‚Ķ26 T‚š’“‚¦‚é—AŒŒ”ńˆĖ‘¶’B¬‚Ķ‚»‚ź‚¼‚ź56.1“C42.6“C5.9“‚Å‚ ‚čC—LˆÓ‚ÉLEN Ž”—Ć‚Ŗ—D‚Į‚Ä‚¢‚½ipƒ0.001jB×–Eˆā“`Šw“I”½‰ž«‚ąLEN 10 mg/“śC5 mg/“śŒQ‚Å‚»‚ź‚¼‚ź50“C25“‚ÉŠĻŽ@‚³‚ź‚½B”’ŒŒ•a‚Ö‚ĢˆŚs‚Ķ3 ŒQŠŌ‚ō·‚Ķ‚Č‚©‚Į‚½1jB—LŠQŽ–Ū‚ąd“Ä‚Č‚ą‚Ģ‚Ķ‚Č‚©‚Į‚½B–{ŽŽŒ±‚Å‚Ķ16 T‚Å‚ĢŽŽŒ±Ž”—ĆŒQƒNƒƒXƒI[ƒo[‚Ŗˆź•”‚Å”F‚ß‚ē‚ź‚Ä‚¢‚½‚ŖC‘S¶‘¶Š„‡iOSj‚Å‚Ķ3 ŒQŠŌ‚É—LˆÓ·‚š”F‚ß‚Č‚©‚Į‚½B

‘“ą‚ĢŽŽŒ±‚Å‚Ķ’įƒŠƒXƒN11 —į‚Ŗ“o˜^‚³‚źCŌŒŒ‹…Œn‰ü‘P‚Ķ‘S—į‚ɁCCyR ‚Ķ3 —į‚É‚Ż‚ē‚ź‚½3jB

del (5q)‚š”ŗ‚ķ‚Č‚¢—AŒŒˆĖ‘¶’įƒŠƒXƒN MDS ‚É‘Ī‚µ‚Ä‚Ķ•Ä‘‚Å‘ęⅡ‘ŠŽŽŒ±‚ŖŽĄŽ{‚³‚źCLEN 10 mg/ “śC21 “ś“Š—^i28 “śƒTƒCƒNƒ‹j‚ĢŒų‰Ź‚ŖŒŸŲ‚³‚ź‚½BŌŒŒ‹…—AŒŒ”ńˆĖ‘¶’B¬—¦‚Ķ26“‚ŁC‚±‚ź‚ē‚ĢĒ—į‚É‚Ø‚Æ‚éƒwƒ‚ƒOƒƒrƒ“ćø’†‰›’l‚Ķ3.2 g/dLC‚»‚ĢŽ‘±ŠśŠŌ‚Ģ’†‰›’l‚Ķ41 T‚Å‚ ‚Į‚½ 4jB‚±‚ĢŒ‹‰Ź‚©‚ēLEN ‚Ŗ del (5q) ‚š”ŗ‚ķ‚Č‚¢ MDS ‚É‘Ī‚µ‚Ĉź’č‚Ģ—LŒų«‚šŽ¦‚·‚±‚Ę‚ŖŠś‘Ņ‚³‚ź‚é‚ŖCŠCŠO‚Å‚Ģ‘ęⅡ‘ŠŽŽŒ±‚Ü‚Å‚ĢŒ‹‰Ź‚µ‚©‚Č‚­C‘“ą‚Å‚ĶŽŽŒ±‚ŖŽĄŽ{‚³‚ź‚Ä‚¢‚Č‚¢i‘“ą“K‰žŠOjB

yŽQl•¶Œ£z

1j List A, et al. Lenalidomide in the myelodysplastic syndrome with chromosome 5q deletion. N Engl J Med. 2006 ; 355i14j : 1456-65.i3iiiDivj

2j Fenaux P, et al. A randomized phase 3 study of lenalidomide versus placebo in RBC transfusion-dependent patients with Low-/Intermediate-1-risk myelodysplastic syndromes with del5q. Blood. 2011 ; 118i14j : 3765-3776.i1iDivj

3j Harada H, et al. Lenalidomide is active in Japanese patients with symptomatic anemia in low- or intermediate-1 risk myelodysplastic syndromes with a deletion 5q abnormality. Int J Hematol. 2009 ; 90i3j : 353-60.i3iiiDivj

4j Raza A, et al. Phase 2 sutdy of lenalidomide in transfusion-dependent, low-risk, and intermediate-1-risk myelodysplastic syndromes with karyotypes other than deletion 5q. Blood. 2008 ; 111i1j : 86-93.i3iiiDivj


CQ6

’įƒŠƒXƒNMDS ‚ĢŽ”—Ć‚Ę‚µ‚ăAƒUƒVƒ`ƒWƒ“‚ĶŠ©‚ß‚ē‚ź‚é‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2B
’įƒŠƒXƒNMDS ‚É‘Ī‚µ‚ăAƒUƒVƒ`ƒWƒ“‚É‚ę‚č‘¢ŒŒ‚Ģ‰ń•œ‚Ķ”F‚ß‚ē‚ź‚é‚ŖC¶‘¶ŠśŠŌ‰„’·‚š–Ś“I‚Ę‚µ‚½‘ęˆź‘I‘š–ņ‚Ę‚µ‚Ä‚ĢŽg—p‚Ķ„§‚³‚ź‚Č‚¢B

y‰š ąz

ƒAƒUƒVƒ`ƒWƒ“iAZAj‚Ķ‚ƒŠƒXƒNMDS ‚É‘Ī‚·‚é‘ę‡V‘Š—Տ°ŽŽŒ±‚Ő¶‘¶‰„’·Œų‰Ź‚ŖŽ¦‚³‚ź‚Ä‚¢‚é1jB‚»‚ĢŽŽŒ±‚Å‚Ķ60“‚Ģ—į‚Å‘¢ŒŒ‰ń•œ‚ŖŠĻŽ@‚³‚ź‚½B’įƒŠƒXƒN‚Ģ‚Ż‚š‘ĪŪ‚Ę‚µ‚½AZA ‚ĢŽŽŒ±‚Ķ‚Č‚¢‚ŖC74 —į‚Ģ’įƒŠƒXƒNMDS ‚É‘Ī‚·‚éŒć•ūŽ‹“I‰šĶ‚Å‚ĶAZA Ž”—Ć‚É‚ę‚Į‚Ä45.9“‚Ģ—į‚Å‘¢ŒŒ‰ń•œ‚ŖŠĻŽ@‚³‚ź‚½2jB‚»‚Ģ’†‚Å4 ƒR[ƒXˆČć‚ĢŽ”—Ć‚šŽó‚Æ‚½64 —į‚Å‚Ķ51.6“‚Ģ”½‰ž‚Ŗ“¾‚ē‚źCŽ”—Ć”½‰ž‚š“¾‚ē‚ź‚½—į‚Ķ”ń”½‰ž—į‚Ę”äŠr‚µ‚摶ŠśŠŌ‚Ŗ‰„’·‚µ‚Ä‚¢‚½B

‘O•ūŽ‹“IŽŽŒ±‚Å‚ĢŒŸ“¢‚Ķ—Տ°ŽŽŒ±‚Ģ•”•Ŗ‰šĶ‚Ģ‚Ż‚Å‚ ‚čC¶‘¶‚ÉŠÖ‚·‚鉚Ķ‚Ķ‚Č‚¢B

Œ»Žž“_‚Å’įƒŠƒXƒNMDS ‚Ģ¶‘¶ŠśŠŌ‰„’·‚š–Ś“I‚Ę‚µ‚½‘ęˆź‘I‘š–ņ‚Ę‚µ‚Ä‚ĢŽg—p‚Ķ„§‚³‚ź‚Č‚¢B

yŽQl•¶Œ£z

1j Fenaux P, et al. Efficacy of azacitidine compared with that of conventional care regimens in the treatment of higher-risk myelodysplastic syndromes : a randomised, open-label, phase Ⅲ study. Lancet Oncol. 2009 ; 10i3j : 223-32.i1iiAj

2j Musto P, et al. Azacitidine for the treatment of lower risk myelodysplastic syndromes : a retrospective study of 74 patients enrolled in an Italian named patient program. Cancer. 2010 ; 116 i6j : 1485-94.i3iiiDivj


CQ7

MDS ‚É‘Ī‚·‚é“ÆŽķ‘¢ŒŒŠ²×–EˆŚA‚Ģ“K‰ž‚Ę“KŲ‚ČŽĄŽ{ŽžŠś‚Ķ‚¢‚Ā‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2A
‚ƒŠƒXƒNMDS Š³ŽŅ‚Å‚ĶC‚Å‚«‚éŒĄ‚葬‚ā‚©‚É“ÆŽķ‘¢ŒŒŠ²×–EˆŚA‚šs‚¤BHLA 1 Ą•s“K‡ˆČ“ą‚ĢŒŒ‰ŽŅŠŌˆŚA‚ŖÅ‚ą–]‚Ü‚µ‚¢‚ŖCŒŒ‰ƒhƒi[‚Ŗ“¾‚ē‚ź‚Č‚¢ź‡‚ĶHLA ˆź’v”ńŒŒ‰ŽŅŠŌˆŚA‚ąl—¶‚·‚éB

„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2A
’įƒŠƒXƒNMDS Š³ŽŅ‚Å‚Ķ“ÆŽķ‘¢ŒŒŠ²×–EˆŚA‚Ķ„§‚³‚ź‚Č‚¢B‚µ‚©‚µCƒŠƒXƒN‚Ģˆ«‰»‚Ü‚½‚Ķˆ«‰»ŒXŒü‚Ŗ‚ ‚éĒ—įC‚“x‚Ģ—AŒŒˆĖ‘¶—įCŒJ‚č•Ō‚µŠ“õĒ‚Ŗ‚Ż‚ē‚ź‚é—įC–ʉu—}§—Ć–@‚Č‚Ē‘¼‚ĢŽ”—Ć–@‚É”½‰ž‚Ŗ‚Ż‚ē‚ź‚Č‚¢Ē—į‚Ķ‘¢ŒŒŠ²×–EˆŚA‚ĢŒó•ā‚Ę‚Č‚éB

„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2A
ä`‘ŃŒŒˆŚA‚Ķ„§‚³‚ź‚Č‚¢‚ŖCœ‘E––½ŒŒƒhƒi[‚Ŗ“¾‚ē‚ź‚Č‚¢‚ƒŠƒXƒNMDS Š³ŽŅ‚Å‚Ķl—¶‚³‚ź‚éB

y‰š ąz

MDS ‚É‚Ø‚¢‚ÄŽ”–ü‚ŖŠś‘Ņ‚Å‚«‚鎔—Ć‚ĶCŒ»Ż‚Ģ‚Ę‚±‚ė“ÆŽķ‘¢ŒŒŠ²×–EˆŚA‚Ģ‚Ż‚Å‚ ‚éB

‚ƒŠƒXƒNMDS ‚Å‚ĶŒŒ‹…Œø­‚ā”’ŒŒ•a‚Ö‚Ģi“WƒŠƒXƒN‚Ŗ‚‚­C‚»‚Ģ‚Ü‚Ü‚Å‚Ķ—\Œć•s—Ē‚Å‚ ‚éB‚±‚Ģ‚½‚߁C“ÆŽķ‘¢ŒŒŠ²×–EˆŚA‚Ŗ‰Ā”\‚Å‚ ‚ź‚ĪŒ“‘„‚Ę‚µ‚Ä‘¬‚ā‚©‚É‚±‚ź‚šŽĄŽ{‚·‚éi•\1jB55 Ī–¢–ž‚ĢŠ³ŽŅ‚ÅHLA ŒŒ“Šw“I1 Ą•s“K‡ˆČ“ą‚ĢŒŒ‰ƒhƒi[‚Ŗ‘¶Ż‚µCˆŚA‚É‘Ļ‚¦‚ē‚ź‚é‘Sgó‘Ō‚ĢĒ—į‚ŖÅ‚ą—Ē‚¢“K‰ž‚Ę‚³‚źC“ś–{‘¢ŒŒŠ²×–EˆŚAŠw‰ļƒf[ƒ^i•½¬23 ”N“xj‚É‚ę‚ź‚ĪC16 ĪˆČć‚ĢRAEB/RAEB-t Ē—į‚ɂ؂Ƃ錌‰ŽŅŠŌœ‘ˆŚA‚Ģ5 ”N‘S¶‘¶Š„‡iOSj‚Ķ50“‚Å‚ ‚é1jBŒŒ‰ŽŅƒhƒi[‚Ŗ‘¶Ż‚µ‚Č‚¢ź‡‚Ķ”ńŒŒ‰ŽŅŠŌˆŚA‚Ŗs‚ķ‚ź‚é‚ŖC“ƃf[ƒ^‚É‚ę‚é‚Ę16 ĪˆČć‚ĢRAEB/RAEB-t Ē—į‚É‚Ø‚Æ‚é”ńŒŒ‰œ‘ˆŚA‚Ģ5 ”NOS ‚Ķ42“‚Ę•ń‚³‚ź‚Ä‚Ø‚čCˆź’č‚Ģ’·Šś¶‘¶‚Ŗ”F‚ß‚ē‚ź‚Ä‚¢‚é1jB

•\1@“ś–{‘¢ŒŒ×–EˆŚAŠw‰ļƒKƒCƒhƒ‰ƒCƒ“‚É‚ę‚éMDS ‚É‘Ī‚·‚éˆŚA“K‰ži”²ˆj3j
IPSSiriskj •aŒ^ HLA “K‡“Æ–E HLA “K‡”ńŒŒ‰ ä`‘ŃŒŒˆŚA–3
Low RA/RARS–1 CO CO Dev
Intermediate-1 RA/RCMD/RS–1 CO CO Dev
RAEB-1–1 CO CO Dev
Intermediate-2 RA/RCMD/RAEB-1 S S CO
RAEB-2–2 S S CO
High RAEB-1/2–2 S S CO

SF standard of careCˆŚA‚Ŗ•W€Ž”—Ć‚Å‚ ‚éi‡•¹ĒCQOL ‚Č‚Ē‚Ģ•s—˜‰v‚É‚Ā‚¢‚Ä‚ąŒŸ“¢‚µ‚½ć‚Å‘‡“I‚ÉŒˆ’č‚·‚ׂ«‚Å‚ ‚éjB

COFclinical optionCˆŚA‚šl—¶‚µ‚Ä‚ą‚ę‚¢B

DevFdevelopmentalCŠJ”­’†‚Å‚ ‚čC—Տ°ŽŽŒ±‚Ę‚µ‚ÄŽĄŽ{‚·‚ׂ«B

–1 ŒŒ‹…Œø­‚“x‚ÅŒŒ‰t•ā[—Ć–@ˆĖ‘¶«‚ ‚é‚¢‚ĶdĒŠ“õĒEoŒŒƒnƒCƒŠƒXƒN‚ĢĒ—į‚ŁC‘¼‚Ģ•Ū‘¶“IŽ”—Ć–@–³Œų‚Ģź‡B

–2 õF‘ĢˆŁķ‚Ŗgood prognosis ‚šŽ¦‚·ˆź•”‚ĢĒ—į‚Å‚ĶˆŚA“K‰ž‚šTd‚ɍl—¶‚·‚éB

–3 Š³ŽŅ”N—īCä`‘ŃŒŒ×–E”‚Č‚Ē‚É‚ę‚čCO ‚Ü‚½‚ĶDev ‚Ę‚Č‚éB

’įƒŠƒXƒNMDS ‚Å‚Ķ‚»‚Ģ‚Ü‚Ü‚Å‚ą”äŠr“I’·Šś‚Ģ¶‘¶‚ŖŠś‘Ņ‚Å‚«‚邽‚߁C‘¢ŒŒŠ²×–EˆŚA‚Ģ“K‰ž‚ĶTd‚ɍl—¶‚·‚ׂ«‚Å‚ ‚čCŒˆ’f•ŖĶ‚ĢŽč–@‚š—p‚¢‚½ˆŚAŽžŠś‚Ģ‰šĶ‚Å‚ĶC‘Ū—\Œć—\‘ŖƒXƒRƒAƒŠƒ“ƒOƒVƒXƒeƒ€iIPSSjLow`Intermediate-1 ‚ĢĒ—į‚Ķ•aŠś‚Ŗis‚µ‚Ä‚©‚ē‚ĢˆŚA‚Ģ•ū‚Ŗ–]‚Ü‚µ‚¢‚±‚Ę‚ŖŽ¦‚³‚ź‚Ä‚¢‚é2jB‚µ‚©‚µC—AŒŒ‚“xˆĖ‘¶CŠ“õĒ‚Ģ”½•œ‚Č‚Ēœ‘•s‘SĒó‚ŖŒ°Ż‰»‚µ‚Ä‚¢‚銳ŽŅ‚Å‚Ķ‡•¹Ē‚É‚ę‚鎀–S‚ā¶Šˆ‚ĢŽæiQOLj‚Ģ’˜‚µ‚¢’į‰ŗ‚Ŗ—\‘z‚³‚ź‚邽‚߁C\•Ŗ‚Č“ÆˆÓ‚š“¾‚½ć‚ňŚA‚šl—¶‚·‚邱‚Ę‚Ķ‰Ā”\‚Å‚ ‚éB

ä`‘ŃŒŒˆŚA‚ĶŽ{s—į”‚Ŗ­‚Č‚­•]‰æ‚Ŗ•s\•Ŗ‚Å‚ ‚čCŒ»’iŠK‚Å‚Ķ•W€Ž”—Ć‚Ę‚µ‚Ä‚Ķ„§‚³‚ź‚Č‚¢BŒŒ‰E”ńŒŒ‰ŽŅœ‘ˆŚAƒhƒi[‚ŖŒ©‚Ā‚©‚ē‚Č‚¢‚©CŽžŠŌ“I—]—T‚Ŗ‚Č‚¢ź‡‚ɗՏ°Œ¤‹†‚Ģ˜g‘g‚Ż‚ōs‚¤‚ׂ«Ž”—Ć‚Ęl‚¦‚ē‚ź‚éB

yŽQl•¶Œ£z

1j “ś–{‘¢ŒŒŠ²×–EˆŚAŠw‰ļ JSHCT ƒf[ƒ^ƒZƒ“ƒ^[C•½¬23 ”N“x‘S‘’²ø•ń‘ :

2j Cutler CS, et al. A decision analysis of allogeneic bone marrow transplantation for the myelodysplastic syndromes : Delayed transplantation for low-risk myelodysplasia is associated with improved outcome. Blood. 2004 ; 104i2j : 579-85.i3iiiAj

3j “ś–{‘¢ŒŒŠ²×–EˆŚAŠw‰ļ@‘¢ŒŒŠ²×–EˆŚAƒKƒCƒhƒ‰ƒCƒ“@œ‘ˆŁŒ`¬ĒŒóŒQi¬ljDJSHCT monograph vol. 18, ƒKƒCƒhƒ‰ƒCƒ“D


CQ8

‚ƒŠƒXƒNMDS ‚É‘Ī‚µ‚ăAƒUƒVƒ`ƒWƒ“‚ĶŠ©‚ß‚ē‚ź‚é‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[1
ƒAƒUƒVƒ`ƒWƒ“‚ĶC“ÆŽķ‘¢ŒŒŠ²×–EˆŚA‚Ŗs‚ķ‚ź‚Č‚¢‚ƒŠƒXƒNĒ—į‚Å‚Ķ ‘ęˆź‘I‘š–ņÜ‚Å‚ ‚éB

„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2B
–¾‚ē‚©‚Č‘ˆ«‚ā—LŠQŽ–Ū‚É‚ę‚é’†Ž~‚šœ‚¢‚āC—LŒų«‚Ģ”»’č‚É‚Ķ­‚Č ‚­‚Ę‚ą4`6 ƒR[ƒX‚ĢŒp‘±‚š—v‚·‚éB

„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2A
ƒAƒUƒVƒ`ƒWƒ“‚ĢˆŚA‘OŽ”—Ć‚Ę‚µ‚Ä‚ĢˆÓ‹`‚ĶŠm—§‚µ‚Ä‚¢‚Č‚¢‚ŖCƒhƒi[ ‚Ģ€”õ‚š‘Ņ‚ĀŠŌ‚Ģ‚Ā‚Č‚¬Ž”—Ɓibridgej‚Ę‚µ‚ÄŽ{s‚šl—¶‚µ‚Ä‚ą‚ę ‚¢B

y‰š ąz

ƒAƒUƒVƒ`ƒWƒ“iAZAj‚ĶDNA ‚ÉŽę‚荾‚Ü‚ź‚ÄDNA ‚Ģƒƒ`ƒ‹‰»‚š—}§‚·‚邱‚Ę‚Åˆā“`Žq”­Œ»‚š‰ń•œ‚³‚¹C‚Ü‚½RNA ‚ÉŽę‚荾‚Ü‚ź‚邱‚Ę‚Åƒ^ƒ“ƒpƒN‡¬‚š‘jŠQ‚µ‚ÄŽE×–EŒų‰Ź‚šŽ¦‚·B

•Ä‘‚É‚Ø‚Æ‚éAZA ‚ĘŽxŽ—Ć–@‚Ģ”äŠrŽŽŒ±iCALGB9221 ŽŽŒ±j‚Å‚ĶMDS ‚Ģ‚·‚ׂĂĢ•aŒ^‚É‚Ø‚¢‚Ä”’ŒŒ•a‰»‚Ŗ’x‚źiAZA 21 ƒJŒŽvs ŽxŽ—Ć–@13 ƒJŒŽjC¶‘¶ŠśŠŌ‚Ŗ‰„’·‚µiAZA 19.9 ƒJŒŽvs ŽxŽ—Ć–@10.5 ƒJŒŽjCQOL ‚Ŗ‰ü‘P‚µ‚½‚±‚Ę‚ŖŽ¦‚³‚ź‚½1jB‚Ü‚½C‰¢•Ä‚ɂ؂Ƃ鍂ƒŠƒXƒNMDS ‚š‘ĪŪ‚Ę‚µ‚½’ŹķŽ”—ƁiŽxŽ—Ć–@C’į—p—Ź‰»Šw—Ć–@C‹­—Ķ‰»Šw—Ć–@j‚Ę‚Ģ‘ęⅢ‘Š”äŠrŽŽŒ±iAZA001 ŽŽŒ±j‚Å‚Ķ¶‘¶ŠśŠŌ‚Ģ‰„’·iAZA 24.5 ƒJŒŽvs ’ŹķŽ”—Ć15.0 ƒJŒŽj‚Ę”’ŒŒ•a‰»‚Ü‚Å‚ĢŠśŠŌ‰„’·iAZA 17.8 ƒJŒŽvs ’ŹķŽ”—Ć11.5 ƒJŒŽj‚ŖŽ¦‚³‚ź‚Ä‚¢‚é2jB

‚ƒŠƒXƒNMDS ‚É‚Ø‚¢‚Ä‚ĶC“ÆŽķ‘¢ŒŒŠ²×–EˆČŠO‚ÉMDS ‚Ģ—\Œć‚š—LˆÓ‚ɉü‘P‚Å‚«‚鎔—Ć–@C–ņÜ‚Ķ‘¶Ż‚µ‚Č‚©‚Į‚½‚½‚߁CˆŚA‚šs‚ķ‚Č‚¢Ē—į‚Å‚ĶAZA ‚Ŗ‘ęˆź‘I‘š–ņ‚ĘˆŹ’u‚Ć‚Æ‚ē‚ź‚éB

–{Ü‚Ģ—LŒų«‚Ķ4 ƒR[ƒX“Š—^‚Ü‚Å‚ÉŒ»‚ź‚邱‚Ę‚Ŗ‘½‚¢‚ŖC4 ƒR[ƒXˆČ~‚ÉŒų‰Ź‚ŖŒ»‚ź‚éĒ—į‚ą–ń25“‚Ż‚ē‚ź‚邽‚߁C–¾‚ē‚©‚ČŽ¾Š³‘ˆ«‚ā—LŠQŽ–Ū‚É‚ę‚é’†Ž~‚šœ‚¢‚āC—LŒų«‚Ģ”»’f‚Ķ­‚Č‚­‚Ę‚ą4`6 ƒR[ƒXŽ{s‚µ‚½Œć‚ɍs‚¤•K—v‚Ŗ‚ ‚é3j4jB

AZA ‚šˆŚA‘OŽ”—Ć‚ÉŽg—p‚µ‚½•ń‚Ķ‚ ‚é‚ŖC‚»‚ĢŽ”—ĆŒų‰ŹEˆÓ‹`‚Ķ–¾‚ē‚©‚É‚Č‚Į‚Ä‚¢‚Č‚¢BˆŚA‘OŽ”—Ć‚Ę‚µ‚ÄŽg—p‚Ķ‰Ā”\‚¾‚ŖCŒų‰Ź”­Œ»‚Ü‚ÅŽžŠŌ‚Ŗ‚©‚©‚邱‚Ę‚šl—¶‚µ‚ÄŽ”—ĆŒv‰ę‚š—§‚Ä‚é•K—v‚Ŗ‚ ‚éB

yŽQl•¶Œ£z

1j Silverman LR, et al : Randomized controlled trial of azacitidine in patients with the myelodysplastic syndrome : A study of the cancer and leukemia group B. J Clin Oncol. 2002 ; 20i10j : 2429-40.i1iiAj

2j Fenaux P, et al : Efficacy of azacitidine compared with that of conventional care regimens in the treatment of higher-risk myelodysplastic syndromes : A randomized, open-label, phase Ⅲ study. Lancet Oncol. 2009 ; 10i3j : 223-32.i1iiAj

3j Silverman LR, et al. : Further analysis of trials with azacitidine in patients with myelodysplastic syndrome : studies 8421, 8921, and 9221 by the Cancer and Leukemia Group B. J Clin Oncol. 2006 ; 24i24j : 3895-903.i3iiDivj

4j Gotze K, et al : Azacitidine for treatment of patients with myelodysplastic syndromes iMDSj : Practical recommendations of the German MDS Study Group. Ann Hematol. 2010 ; 89i9j : 841-50.


CQ9

‚ƒŠƒXƒNMDS ‚É‘Ī‚µ‚ăŒƒiƒŠƒhƒ~ƒh‚ĶŠ©‚ß‚ē‚ź‚é‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2B
‚ƒŠƒXƒNMDS ‚É‘Ī‚·‚郌ƒiƒŠƒhƒ~ƒh‚ĢŽg—p‚Ķ„§‚³‚ź‚Č‚¢B

„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2B
del (5q) ‚š—L‚·‚é MDS ‚É‚Ø‚¢‚Ä‚ĶCƒAƒUƒVƒ`ƒWƒ“•s‰ž‚Ģź‡CƒŒƒiƒŠƒhƒ~ƒh‚ĢŽg—p‚šl—¶‚µ‚Ä‚ą‚ę‚¢B

y‰š ąz

Œ»Žž“_‚ō‚ƒŠƒXƒNMDS ‚ɂ؂Ƃ郌ƒiƒŠƒhƒ~ƒhiLENj‚Ģ—L—p«‚š–¾Šm‚ɏŲ–¾‚µ‚½ƒGƒrƒfƒ“ƒX‚Ķ‚Č‚­C‚ƒŠƒXƒNMDS ‚Ö‚ĢŽg—p‚Ķ„§‚³‚ź‚Č‚¢B

‚µ‚©‚µCdel (5q) ‚š—L‚·‚鍂ƒŠƒXƒN MDS 47—į‚š‘ĪŪ‚Ę‚µ‚½—Տ°ŽŽŒ±‚É‚Ø‚¢‚āC13 —įi27“j‚ÉŒŒ‰tŠw“I‰ü‘P‚Ŗ”F‚ß‚ē‚źm7 —įi15“j‚ĶŠ®‘S‘tŒųnC5q ’P“ĘŒ‡‘¹9 —į’†6 —į‚ĶŠ®‘S‘tŒų‚É“ü‚Į‚½‚±‚Ę‚Ŗ•ń‚³‚ź‚Ä‚¢‚éBƒAƒUƒVƒ`ƒWƒ“iAZAj•s‰ž‚Ģ del (5q) MDS ‚Å‚Ķ LEN ‚ĶŒó•ā–ņ‚Ę‚µ‚čl—¶‚³‚ź‚é1jB

yŽQl•¶Œ£z

1j Ades L, et al : Efficacy and safety of lenalidomide in intermediate-2 or high-risk myelodysplastic syndromes with 5q deletion : results of a phase 2 study. Blood. 2009 ; 113i17j : 3947-52.i3iiiDivj


CQ10

‚ƒŠƒXƒNMDS ‚É‚Ø‚¢‚ĉ»Šw—Ć–@‚ĶŠ©‚ß‚ē‚ź‚é‚©


„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2A
¶‘¶ŠśŠŌC”’ŒŒ•a‰»‚Ü‚Å‚ĢŠśŠŌ‚š‰„’·‚·‚鉻Šw—Ć–@‚Ģ•ń‚Ķ‚Č‚­C‘ęˆź‘I‘š‚Ę‚µ‚Ä‚Ķ„§‚³‚ź‚Č‚¢iƒAƒUƒVƒ`ƒWƒ“‚Ŗ„§‚³‚ź‚éjB

„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2B
“ÆŽķ‘¢ŒŒŠ²×–EˆŚA‚ŖŽĄŽ{‚³‚ź‚Č‚¢Žį”N—į‚ŐõF‘ĢˆŁķC‘Sgó‘ŌiPSjCœė•aŠśŠŌ‚Č‚Ē‚Ģ—\Œć•s—ĒˆöŽq‚Ģ‚Č‚¢Ē—į‚Å‚Ķ‹­—Ķ‰»Šw—Ć–@‚ąŒó•ā‚Ę‚Č‚é‚ŖC‰»Šw—Ć–@‚ĶƒAƒUƒVƒ`ƒWƒ“‚ŖŽg—p‚Å‚«‚Č‚¢ź‡‚É“K‰ž‚Ŗl—¶‚³‚ź‚éB

„§ƒOƒŒ[ƒh
ƒJƒeƒSƒŠ[2B
‹­—Ķ‰»Šw—Ć–@‚Ę’į—p—Ź‰»Šw—Ć–@‚Ģ¶‘¶ŠśŠŌ‚Ö‚Ģ‰e‹æ‚Ķ‚Ł‚Ś“Æ“™‚Å‚ ‚čC‚»‚Ģ“K‰ž‚ĶĒ—į‚²‚Ę‚É”»’f‚·‚éB

y‰š ąz

]—ˆC‚ƒŠƒXƒNMDS ‚É‘Ī‚µ‚Ä‚Ķ‰»Šw—Ć–@‚Ŗs‚ķ‚ź‚Ä‚«‚½‚ŖCˆź•”Žį”N—į‚ŁCõF‘ĢˆŁķCPSCœė•aŠśŠŌ‚Č‚Ē‚Ģ—\Œć•s—ĒˆöŽq‚Ģ‚Č‚¢Ē—į‚Å‚Ķ‹­—Ķ‰»Šw—Ć–@‚Ģ—L—p«‚ŖŽ¦‚³‚ź‚Ä‚¢‚é‚ą‚Ģ‚Ģ1jC‚»‚źˆČŠO‚ĢĒ—į‚Å‚Ķ¶‘¶ŠśŠŌ‚ā”’ŒŒ•a‰»‚Ü‚Å‚ĢŠśŠŌ‰„’·‚š–¾Šm‚ÉŽ¦‚µ‚½ƒŒƒWƒƒ“‚Ķ‘¶Ż‚µ‚Č‚¢B‚±‚Ģ‚½‚߁C‰»Šw—Ć–@‚Ģ“K‰ž‚ĶƒAƒUƒVƒ`ƒWƒ“iAZAj‚ŖŽg—p‚Å‚«‚Č‚¢Ē—įi•s‰žE•s‘Ļj‚ɍl—¶‚³‚ź‚éB

MDSiRAEB-t ‚Ø‚ę‚Ń”’ŒŒ‰»Ē—į‚šŠÜ‚Žj‚É‚Ø‚Æ‚é‹­—Ķ‰»Šw—Ć–@‚Ę’į—p—Ź‰»Šw—Ć–@‚š”äŠr‚µ‚½‚ķ‚Ŗ‘‚Ģ—Տ°ŽŽŒ±iJALSG MDS200 ŽŽŒ±j‚Å‚ĶC“o˜^Ē—į”‚Ŗ•s\•Ŗ‚Å“ŒvŠw“I‚Č”äŠr‚Ŗ‚Č‚³‚ź‚Ä‚¢‚Č‚¢‚ą‚Ģ‚ĢCŠ°‰š—¦‚Å‚Ķ‹­—Ķ—Ć–@ŒQ‚Ŗ‚‚©‚Į‚½‚É‚ą‚©‚©‚ķ‚ē‚øi‹­—Ķ‰»Šw—Ć–@64.7“ vs ’į—p—Ź‰»Šw—Ć–@43.9“jC2 ”N–³•a¶‘¶Š„‡iDFSj‚Ø‚ę‚Ń2 ”N‘S¶‘¶Š„‡iOSj‚Å‚Ķ‚Ł‚Ś“Æ“™‚Å‚ ‚čiDFSG‹­—Ķ‰»Šw—Ć–@26.0“ vs ’į—p—Ź‰»Šw—Ć–@24.8“COSG‹­—Ķ‰»Šw—Ć–@28.1“ vs ’į—p—Ź‰»Šw—Ć–@32.1“jCMDS ‚Å‚ĶŠ°‰š“±“ü—¦‚Ŗ—\Œć‚š•K‚ø‚µ‚ą”½‰f‚¹‚øC‹­—Ķ‰»Šw—Ć–@‚Ę’į—p—Ź‰»Šw—Ć–@‚Ķ‚Ł‚Ś“Æ“™‚Ģ¬Ń‚Å‚ ‚Į‚½2jB

yŽQl•¶Œ£z

1j Kantarjian H, et al : Long-term follow-up results of the combination of topotecan and cytarabine and other intensive chemotherapy regimens in myelodysplastic syndrome. Cancer. 2006 ; 106 i5j : 1099-109.i3iiiDivj

2j Morita Y, et al : Comparative analysis of remission induction therapy for high-risk MDS and AML progressed from MDS in the MDS200 study of Japan Adult Leukemia Study Group. Int J Hematol. 2010 ; 91i1j : 97-103.i1iiDij