伴fms样酪氨酸激酶3基因内部串联重复突变的急性早幼粒细胞白血病患者初次诱导临床特点研究  

Clinical characteristics of initial induction of acute promyelocytic leukemia patients with fms-like tyrosine kinase 3-internal tandem duplication mutation

作　　者：唐宇宏[1] 王育丽[1] 司阳 路伟 张彦芳 TANG Yuhong;WANG Yuli;SI Yang;LU Wei;ZHANG Yanfang(Department of Hematology,Shanghai Ninth People’s Hospital,Shanghai Jiao Tong University School of Medicine,Shanghai 201900,China)

机构地区：[1]上海交通大学医学院附属第九人民医院血液科,上海201900

出　　处：《上海医学》2021年第6期405-408,共4页Shanghai Medical Journal

基　　金：国家自然科学基金(81900145);上海市科技人才计划项目(18YF1419100)。

摘　　要：目的通过研究fms样酪氨酸激酶3(fms-like tyrosine kinase 3,FLT3)基因内部串联重复(internal tandem duplication,ITD)突变的急性早幼粒细胞白血病(acute promyelocytic leukemia,APL)患者初次诱导过程中的临床特征,为探索FLT3-ITD突变的APL患者更有效的治疗提供思路。方法回顾性分析18例初发APL患者的临床资料,将患者分为基因突变组(8例,其中2例在诱导中死亡)和非基因突变组(10例)。观察两组患者诱导前的白细胞(white blood cell,WBC)计数、诱导中WBC计数峰值、维甲酸综合征(retinoic acid syndrome,RAS)发生率、弥散性血管内凝血(disseminated intravascular coagulation,DIC)发生率,以及完全缓解(complete response,CR)所需时间等指标。结果基因突变组与非基因突变组诱导前WBC计数分别为16.73(1.30,20.30)×10^(9)/L和2.94(0.60,3.70)×10^(9)/L,两组间差异有统计学意义(P<0.05)。WBC计数峰值分别为(39.21±14.28)×10^(9)/L和(28.15±19.40)×10^(9)/L,达峰时间分别为(8.5±2.5)和(4.8±2.0)d,两组间差异均无统计学意义(P值均>0.05);RAS发生率分别为4/6和3/10;DIC发生率分别为4/8和2/10;达到CR时间分别为(30.0±1.63)和(27.6±6.7)d,两组间差异无统计学意义(P>0.05)。结论FLT 3-ITD突变的APL患者在初次诱导治疗过程中不良事件发生率高、程度重,提示FLT 3-ITD是APL预后不良因素。Objective To study the clinical characteristics of acute promyelocytic leukemia(APL)patients with fms-like tyrosine kinase 3-internal tandem duplication(FLT3-ITD)mutations during the initial induction process and explore the treatment of FLT3-ITD mutation-positive APL patients.Methods Clinical data of 18 patients with newly diagnosed APL were retrospectively analyzed.The patients were divided into FLT3-ITD mutation group(n=8,2 patients in the FLT3-ITD mutation group died in induction of remission)and non-mutation group(n=10).The white blood cell(WBC)count before induction,the peak value of WBC after induction,retinoic acid syndrome(RAS)incidence and duration,disseminated intravascular coagulation(DIC)incidence,complete response(CR)rate and time required were observed.Results FLT3-ITD mutation group had more WBC than FLT3-ITD non-mutation group before induction[16.73(1.30,20.30)×10^(9)/L vs.2.94(0.60,3.70)×10^(9)/L,P<0.05].There was no significant difference in the peak value of WBC after induction or the time to peak between FLT3-ITD mutation group and FLT3-ITD non-mutation group[(39.21±14.28)×10^(9)/L vs.(28.15±19.40)×10^(9)/L,(8.5±2.5)d vs.(4.8±2.0)d,both P>0.05].The incidence of RAS was 4/6 in FLT3-ITD mutation group and 3/10 in FLT3-ITD non-mutation group.The incidence of DIC was 4/8 and 2/10,and the time to CR was(30.0±1.63)d and(27.6±6.7)d(P>0.05).Conclusion APL patients with FLT3-ITD mutations have a high probability and severity of adverse events during the initial induction of remission,suggesting that FLT3-ITD is a poor prognostic factor for APL.

关 键 词：fms样酪氨酸激酶3基因 内部串联重复 突变 急性早幼粒细胞白血病 维甲酸综合征 弥散性血管内凝血 

分 类 号：R733.71[医药卫生—肿瘤]