【摘要】 目的分析伴有CEBPA双位点突变的急性髓系白血病(AML)的临床特征及预后。方法回顾性分析2013年1月至2019年5月浙江大学医学院附属第一医院及丽水市人民医院血液科连续收治的AML(非M3)患者1030例,其中CEBPA双位点突变AML共75例为试验组,非CEBPA双位点突变AML共955例为对照组。比较两组患者的临床特征及预后差异。结果试验组AML患者FAB分型中M4比例明显高于对照组(P=0.004);试验组初诊PLT水平低于对照组(P=0.041);试验组预后中等染色体核型比例明显高于对照组(P=0.001);试验组CD7抗原阳性比例明显高于对照组(P<0.001);试验组患者早期死亡率为4.00%,低于对照组患者的13.10%(P=0.022),其预计5年总生存(OS)率明显高于对照组(57.33%vs. 30.99%,P<0.001);试验组中多因素分析显示,年龄≥60岁、HGB<88.75 g/L和不良染色体核型是预后不良的独立危险因素。结论试验组AML患者因有较低的早期死亡率,其预后明显优于对照组患者;年龄≥60岁、HGB<88.75 g/L和不良染色体核型是CEBPA双位点突变AML患者预后不良的独立危险因素。更多还原

【Abstract】 Objective To analyze the clinical features and prognosis of acute myeloid leukemia(AML) with double CEBPA mutations. Methods A retrospective analysis was made on 1030 consecutive AML(non-M3) patients admitted to the First Affiliated Hospital of Zhejiang University School of Medicine and the hematology department of Lishui Municipal People′s Hospital from January 2013 to May 2019, of which CEBPA double-site mutant AML(n=75) were the experimental group and non-CEBPA double-site mutant AML(n=955) were the control group. The clinical features and prognostic analysis of the two groups were compared. Results The proportion of M4 in FAB typing of AML patients in the experimental group was significantly higher than that in the control group(P=0.004). The PLT level in the experimental group was lower than that in the control group(P=0.041). The proportion of moderate chromosome karyotype in the experimental group was significantly higher than that in the control group(P=0.001). The positive rate of CD7 antigen in the experimental group was significantly higher than that in the control group(P<0.001). The early mortality rate of the patients in the experimental group was 4.00%, which was lower than that of the control group(13.10%)(P=0.022),and its estimated 5-year overall survival(OS) rate was significantly higher than that of the control group(57.33% vs30.99%, P <0.001). Multivariate analysis in the experimental group showed that age ≥60 years old, HGB <88.75 g/L and poor karyotype were independent risk factors for poor prognosis. Conclusion The prognosis of AML patients in the experimental group is obviously better than that in the control group due to their lower early mortality rate. Age ≥60 years old, HGB<88.75 g/L and poor chromosome karyotype are independent risk factors for poor prognosis of CEBPA double site mutant AML patients.更多还原