Evi1基因阳性急性髓细胞白血病14例临床分析

Clinical analysis of 14 acute myeloid leukemia patients with Evi1 gene positive

目的：分析Evi1基因阳性急性髓细胞白血病（AML）患者的临床特征以及预后特点。方法：收集14例Evi1基因阳性AML患者的临床以及实验室资料,其中男、女各7例,中位年龄49（31~67）岁,初诊时白细胞计数（27.50±41.61）×109/L,血红蛋白（75.50±20.92）g/L,血小板计数（42.86±46.73）×109/L,骨髓原始细胞百分比（49.60±23.30）%。3例由骨髓增生异常综合征（MDS）转化而来,伴有病态造血的比例为42.86%（6/14）,表现为髓系形态异常。存在染色体异常的比例为22.22%（2/9）,其中伴有3号染色体异常1例,7号染色体缺失1例。结果：14例患者中12例进行诱导化疗,5例（41.67%）获得完全缓解,患者中位生存时间为122.5（10~562）d,12个月总体生存率为47.9%,18个月总体生存率为16.0%。结论：Evi1基因阳性AML常常伴有不同程度的病态造血,且与MDS转化有关,其治疗后的完全缓解率低,总体生存期短,预后差。

Objective：To analyze the clinical characters and prognostic features of acute myeloid leukemia patients with Evi1 gene positive.Method：We included 14 adult AML patients（7 males and 7 females,aged 31 to 67years,median 49years）with Evi1 gene positive.At diagnosis,WBC was（27.50±41.61）×109/L,HGB was（75.50±20.92）g/L,PLT was（42.86±46.73）×109/L,bone marrow blast was（49.60±23.30）%.Three patients were transformed from MDS,and proportion with dysplasia was 42.86%（6/14）,which mainly manifested myeloid morphologic abnormalities.The proportion of parents with chromosomal abnormalities was 22.22%（2/9）,one with chromosome 3 abnormality and the other with missing chromosome 7.Result：Twelve of 14 patients received induction chemotherapy,and the complete remission rate was 41.67%（5/12）.The median survival time was 122.5（10to 562）days.One-year overall survival rate was 47.9% and 18 months overall survival rate was 16.0%.Conclusion：Evi1gene positive AML patients have high proportion dysplasia and relate to MDS transformation,besides the patients have low complete remission,short overall survival and poor prognosis.