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急性早幼粒细胞白血病 (APL) 诊疗指南. 急性早幼粒细胞白血病 (APL) 诊疗指南. 第一部分 初诊患者入院检查、诊断. 1 .病史采集及重要体征. 年龄. 1. 此前有无血液病史(主要指 MDS 、 MPN 等). 2. 是否为治疗相关性 ( 包括肿瘤放疗、化疗 ). 3. 有无重要脏器功能不全 ( 主要指心、肝、肾功能). 4. 有无髓外浸润 ( 主要指中枢神经系统白血病 ﹝CNSL﹞). 5. 2 .实验室检查. 血常规、血生化、出凝血检查. 实验室 检查. 免疫分型. 细胞遗传学 t(15;17). - PowerPoint PPT Presentation
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(APL)
(APL)
12345MDSMPN()((CNSL)1
()t(15;17) PML-RAR(PLZF-RARNuMA-RARNPM-RARStat5bRAR)FLT3-ITD2
3.APLt(15;17)PML-RARAPL (APLPLZF-RARNuMA-RARNPM-RARStat5bRAR )APL
(APL) (APL)
APL*/(WBC10 109/L)(WBC10109/L)ATRAATO a* ATRA72ATRA
ATRAATO6 Allo-HSCTAPL-2APL-3APL-1(APL)APLt1517PML-RAR
a ATRA 20mg/m2/d po ATO 0.16mg/kg/d ivgtt
* 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-HSCTAPL-2a 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
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-4APL-3(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
LPA 96 PFSLPA 96 EFS according to presenting WBC countWBCLPA 96_Blood_1999
LPA 96LPA 99LPA96LPA99AIDAATRA+IDALPA96LPA99ATRA
MTZ 1LPA992IDAIDALPA 99_Blood_2008
LPA 96LPA 99DFSCIRLPA995CIRDFS11%84%LPA 96P 0 .017 0.03LPA 99_Blood_2008
--LPA 96LPA 99LPA2005LPA2005LPA991240%LPA 2005_Blood_2010
LPA 2005LPA2005CIRP=0.03
LPA99LPA2005OSDFSCIRP
LPA 2005_Blood_2010
LPA99LPA2005CIR4CIR LPA99 27% LPA2005 14% P=0.03LPA 2005_Blood_2010
LPA99LPA2005DFSOSLPA2005DFS (P=.04) P.001 P=.04 LPA 2005_Blood_2010
AIDA-2000 GIMEMA_Blood_2010
CIRAIDA-2000 GIMEMA_Blood_2010
80ATRAAPLAPLAPLAIDAATRA+IDAAPL ATRA
ARTA+ATO2004ATRAATOAPLCR90%,PML-RARATRA+ATOProc Natl Acad Sci USA 2004;101(15):5328-35.2011NCCN
APL-4(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
European APL 93 EuroAPL93 10ATRA+6mp+MTXBlood.2010;115:1690-1696)
a b4
APLAPL-6a 3-66-12b
APL-5
APL-6APLAPLAPL
APL-7 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
APL-8