急性早幼粒细胞白血病 诊疗指南解读
description
Transcript of 急性早幼粒细胞白血病 诊疗指南解读
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APL10% - 30% ()
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12345MDSMPN()((CNSL)1
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()t(15;17) PML-RAR(PLZF-RARNuMA-RARNPM-RARStat5bRAR)FLT3-ITD2
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---10% 80% 15~20% t15;17 PMLRAR t11;17 PLZFRAR, NuMA RAR t5 ;17 NPM RAR
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MPO++CD13++CD33++HLA-DR-/+CD34-/+CD117-/+CD15-/+CD11b-CD56-/+ CD56(+)APL
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APLAL / ALCD33 CD56 HLA-DR CD16 IgFc t1517M3vATRACT
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APLt(15;17)PML-RARAPL (APLPLZF-RARNuMA-RARNPM-RARStat5bRAR )APL
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APL*/(WBC10 109/L)(WBC10109/L)ATRAATO a* ATRA72ATRA
ATRAATO6 Allo-HSCTAPL-2APL-3(APL)APLt1517PML-RAR
a ATRA 20mg/m2/d po ATO 0.16mg/kg/d ivgtt
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* 46
PCR
/(WBC10 109/L)ATRA+ IDA/DNR+ ATO aATRA + IDA/DNR a* APL-4 Allo-HSCT
*ATRAb + IDA 8-12mg/m2/dDNR 45-90mg/m2/d3d 2APL-4ATOAllo-HSCTa ATRA 20mg/m2/d poATO 0.16mg/kg/d ivgtt IDA 8-12mg/ m2/d iv2, 4, 6, 8 DNR 45-90mg/ m2/d iv2, 4, 6 8
b ATRA 20mg/ m2/d 14 (APL)ATRAb + IDA 8-12mg/m2/dDNR 45-90mg/m2/d3d 2
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10109/LFLT3-ITDATRA + ATO + IDADNR aATRA + IDA aATRA + DNR (Ara-C) a*ATRAb + IDA 8-12mg/ m2/d DNR 45-90mg/ m2/d 3d+ Ara-C 150mg/ m2/d 7d 2 ATRAb + HHT 4mg/ m2/d 3d + Ara-C 1g/ m2 q12h 3d 1
ATO Allo-HSCT Allo-HSCTAPL-4(APL)a ATRA 20mg/m2/d po ATO 0.16mg/kg/d ivgtt IDA 8-12mg/ m2/d iv 2, 4, 6, 8 DNR 45-90mg/ m2/d iv 2, 4, 6 8 Ara-c 150mg/ m2/d iv 1-7
b ATRA 20mg/ m2/d 14 * 46
PCR
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LPA 96 PFSLPA 96 EFS according to presenting WBC countWBCLPA 96_Blood_1999
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ATRA ATRADA+DX ATRA ATRA+
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ATRAAPL ATRACR ATRA ATRA ATRA II (CRABPII)
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ATRAPML/ RAR Dermine ATRANB4306 t(15;17) 110kd PML/ RAR P- -1(MDR-1) Kizaki ATRA HL-60 APL MDR-1 MDR-1 ATRA
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ATRAATRA 450(CP450) ATRA 4--ATRA10 ATRA RA ATRA RA 411 CT RAR52 ATRA
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RAS CD54 / ICAM-1 IL-1 IL-6 IL-8 TNF- G-CSF CD13 CD13
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RASRASRASRAS
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RAS ATRADX 10mg IVq12h2w
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(APL)/ATRA 20mg/m2/d14d14(1)ATO 0.16mg/kg/d14d14 14d(2-3) 5
ATRA 20mg/m2/d14d14(1)ATOb 0.16mg/kg/d14d1414d(2-3)MTX 15 mg/m2/w 4w6-MP c 50 mg/m2/d 2-4W(3) 5a PCR(PML-RAR)b ATOSFDAAPLc 6-MPd 23PCR a4
dCRAPLAPL-64APL-5
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a b4
APLAPL-6a 3-66-12b
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APLAPL
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APL APL a b() (CNSL)30-50109/LPPSBFG>150mg/dLPTAPTTDIC ATRA(10mg bid 2)/
3 6 >10109/L QTcCa,K,MgK>4mEq/dLMg>1.8mg/dlQTc>500 msAPL
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10%-15%
WBC PLT CD56 CD34CD2 PML/RAR
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APLGIMEMA 13/97APL93 3/75MRC 3/75
CNS
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CNSCNS 8O% CNS5O
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CNS
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CNS Queen Mary HospitalCNSAPLCNS
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80ATRAAPLAPLAPLAIDAATRA+IDAAPL