幼年型粒-单核细胞白血病临床分析

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。

Clinical Analysis of Juvenile Granulocyte-monocyte Leukemia

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.