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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 characteristics and prognosis of Evi1 positive acute myeloid leukemia (AML) patients. Methods: The clinical and laboratory data of 14 AML patients with positive Evi1 gene were collected, including 7 males and 7 females, with a median age of 49 years (range from 31 to 67 years) and a white blood cell count (27.50 ± 41.61) × 109 / L at the time of first visit. Hemoglobin (75.50 ± 20.92) g / L, platelet count (42.86 ± 46.73) × 109 / L and percentage of bone marrow blast cells (49.60 ± 23.30)%. Three cases were transformed from myelodysplastic syndrome (MDS) with morbid hematopoietic proportion of 42.86% (6/14), which showed abnormal myeloid morphology. The proportion of chromosome abnormalities was 22.22% (2/9), of which 1 was associated with chromosomal abnormality on chromosome 3 and 1 on chromosome 7. Results: Among the 14 patients, 12 patients underwent induction chemotherapy and 5 patients (41.67%) achieved complete remission. The median survival time was 122.5 (ranged from 10 to 562 days). The overall survival rate at 12 months was 47.9% Survival rate was 16.0%. CONCLUSION: Evi1 positive AML is often associated with varying degrees of morbid hematopoiesis and is associated with MDS conversion. Its complete remission rate after treatment is low, overall survival is short, and its prognosis is poor.