幼年型粒-单核细胞白血病临床分析

Clinical Analysis of Juvenile Granulocyte-monocyte Leukemia

目的:通过10例幼年型粒单核细胞白血病(JMML)的临床分析阐明该病的临床特点、诊断要点、治疗及预后。方法:对10例JMML患儿的临床资料进行回顾性分析。结果:临床症状中发热7例,肝大8例,脾大9例,皮疹4例,白细胞中位值45.30×109/L,血红蛋白中位值83 g/L,血小板中位值25×109/L,外周血见原始幼稚者8例。8例JMML行HbF检查均增高。10例行JMML相关基因检测,3例阳性。结论:JMML临床以发热、肝脾肿大为主要表现,多有白细胞增高,HbF增高,基因有助于诊断,误诊率高,预后不良,异基因造血干细胞移植为唯一可以治愈本病的方法。

Objective：To clarify clinical features,diagnostic criteria,treatment and prognosis of the disease according to analysis 10 children suffered juvenile myelomonocytic leukemia（JMML）.Method：Clinical data of 10 children with JMML were retrospectively analyzed.Result：Clinical symptoms included 7 cases with fever,8 cases with hepatomegaly, 9 cases with splenomegaly,4 cases with rash,the median white blood cell count was 45.30×109/L,the median hemoglobin was 83 g/L,and the median platelet count was 25×109/L.8 peripheral blood smear had naive cells.HbF tests were performed in 8 cases,all increased.10 cases were given genetic testing,3 cases were positive.Conclusion：JMML Mainly manifest with fever,hepatosplenomegaly,most with increased white blood cells and HbF,genetic testing contribute to the diagnosis,it has a high misdiagnosis rate,and poor prognosis.Allogeneic hematopoietic stem cell transplantation is the only way to cure the disease.