急性早幼粒细胞白血病 诊疗指南解读

Click here to load reader

Upload: ruby

Post on 12-Jan-2016

152 views

Category:

Documents


0 download

DESCRIPTION

急性早幼粒细胞白血病 诊疗指南解读. 嘉兴第一医院血液科 郭晓珺. APL. 发病率占急性白血病的 10% - 30% 病情凶险,易并发出血,早期死亡率高 传统治疗方法是用细胞毒药物 ( 化疗 ) 将白血 病细胞杀伤至最低水平,易引起严重出血而 致死. 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. - PowerPoint PPT Presentation

TRANSCRIPT

  • APL10% - 30% ()

  • 12345MDSMPN()((CNSL)1

  • ()t(15;17) PML-RAR(PLZF-RARNuMA-RARNPM-RARStat5bRAR)FLT3-ITD2

  • ---10% 80% 15~20% t15;17 PMLRAR t11;17 PLZFRAR, NuMA RAR t5 ;17 NPM RAR

  • MPO++CD13++CD33++HLA-DR-/+CD34-/+CD117-/+CD15-/+CD11b-CD56-/+ CD56(+)APL

  • APLAL / ALCD33 CD56 HLA-DR CD16 IgFc t1517M3vATRACT

  • APLt(15;17)PML-RARAPL (APLPLZF-RARNuMA-RARNPM-RARStat5bRAR )APL

  • 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

  • * 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

  • 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

  • LPA 96 PFSLPA 96 EFS according to presenting WBC countWBCLPA 96_Blood_1999

  • ATRA ATRADA+DX ATRA ATRA+

  • ATRAAPL ATRACR ATRA ATRA ATRA II (CRABPII)

  • ATRAPML/ RAR Dermine ATRANB4306 t(15;17) 110kd PML/ RAR P- -1(MDR-1) Kizaki ATRA HL-60 APL MDR-1 MDR-1 ATRA

  • ATRAATRA 450(CP450) ATRA 4--ATRA10 ATRA RA ATRA RA 411 CT RAR52 ATRA

  • RAS CD54 / ICAM-1 IL-1 IL-6 IL-8 TNF- G-CSF CD13 CD13

  • RASRASRASRAS

  • RAS ATRADX 10mg IVq12h2w

  • (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

  • a b4

    APLAPL-6a 3-66-12b

  • APLAPL

  • 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

  • 10%-15%

    WBC PLT CD56 CD34CD2 PML/RAR

  • APLGIMEMA 13/97APL93 3/75MRC 3/75

    CNS

  • CNSCNS 8O% CNS5O

  • CNS

  • CNS Queen Mary HospitalCNSAPLCNS

  • 80ATRAAPLAPLAPLAIDAATRA+IDAAPL