FLT3-ITD阳性与阴性急性髓细胞白血病的实验室特征及预后比较  

Comparison of molecular biology,immunological characteristics and clinical efficacy in patients with acute myelogenous leukemia with or without FLT3-ITD gene mutation

作　　者：李婷婷[1,2] 孙雪冬[2] 董征[2] 余长林[2] 孙琪云[2] 乔建辉[2] 胡锴勋[2] 刘广贤[2] 姚波[2] 艾辉胜[2] 郭梅[2] 

机构地区：[1]安徽医科大学研究生院,合肥230032 [2]军事医学科学院附属医院血液科

出　　处：《解放军医学杂志》2013年第6期501-505,共5页Medical Journal of Chinese People's Liberation Army

基　　金：国家自然科学基金重点项目(81130054);国家自然科学基金面上项目(81170493)~~

摘　　要：目的比较伴FLT3基因突变(FLT3-ITD+)与不伴FLT3基因突变(FLT3-ITD-)的急性髓细胞白血病(AML)患者的实验室特征、疗效以及预后。方法 2010年10月-2012年10月收治的初诊AML患者93例,年龄10~66岁,包括21例FLT3-ITD+AML(22.6%)和72例FLT3-ITD-AML(77.4%,对照组)列入分析。比较两组患者的临床表现,HOX11、EVI、ETO、MLL、NPM1基因表达情况,细胞学抗原(CD34/CD38、淋巴抗原、CD7和CD4)表达情况,细胞遗传学表现(正常核型、复杂核型),2年无事件生存(EFS)和2年总生存(OS)情况。结果 FLT3-ITD+AML初诊时合并外周血白细胞增高(>100×109/L)及出血者多于FLT3-ITD-组(P<0.01,P=0.004),但两组在年龄、免疫表型及染色体异常等方面无明显差异。FLT3-ITD+AML完全缓解(CR)率28.57%,显著低于FLT3-ITD-AML的55.56%(P=0.03)。FLT3-ITD+AML 2年无事件生存率为29.2%,低于FLT3-ITD-AML组(37.7%,P=0.04)。FLT3-ITD+AML联合表达NPM1者占33.33%,明显高于FLT3-ITD-AML组(1.39%,P=0.001)。FLT3-ITD+AML中表达NPM1的患者均未达完全缓解。结论 FLT3-ITD+AML易合并白细胞增高,完全缓解率低,无事件存活率低,预后差。Objective To compare the clinical and laboratory characteristics, therapeutic efficacy and prognosis of acute myelogenous leukemia （AML） with or without FLT3-ITD gene mutation. Methods The clinical data of 93 AML patients, aged from 10 to 66 years, were retrospectively analyzed. Among them, 21 patients were FLT3-ITD gene （FLT3-ITD＋ AML group） positive, and 72 patients were negative for FLT3-ITD gene （FLT3-ITD- AML group）. The patients＇ chromosomes were examined and mutation of FLT3-ITD and NPM1 gene and the expressions of HOX11, ETO, EVI and NPM1 gene were analyzed, along with the 2-year event-free survival and 2-year overall survival were learned. Results At primary diagnosis, the proportion of patients with WBC〉100 × 109/L, and bleeding rate was significantly higher in FLT3-ITD＋ AML group than that in FLT3-ITD- AML group （P=0.04; P=0.01）, but no statistical difference was found in age, immunophenotype and chromosomal abnormality （P〉0.05）. The complete remission （CR） rate was 28.57% in FLT3-ITD＋ AML group, and it was obviously lower than that in FLT3-ITD- AML group （55.56%, P=0.013）. The 2-year event-free survival was 29.2% in FLT3-ITD＋ AML group and it was significantly lower than that in FLT3-ITD- AML group （37.7%; P=0.04）. Meanwhile, 33.3% of the patients in FLT3-ITD- AML group were also positive for NPM1 gene expression, and all of them did not get complete remission. However, only 1.39% of the patients expressed NPM1 gene in FLT3-ITD- AML group, but 50% of them got complete remission （P=0.022）. Conclusion Patients with FLT3-ITD^AML were easily associated with high WBC counts, bleeding, lower CR rate, poor event-free survival, and poor prognosis.

关 键 词：白血病 前髓细胞性 急性 易位 遗传 FLT3基因 NPM1基因 

分 类 号：R733.712[医药卫生—肿瘤]