10例幼年型粒单核细胞白血病临床分析

Clinical characteristics of juvenile myelomonocytic leukemia in 10 cases

目的:了解幼年型粒单核细胞白血病(JMML)的临床特征及诊断要点。方法:回顾性分析10例JMML患儿的临床及实验室资料。结果:1JMML多见于男孩,以发热、皮肤出血、腹部膨隆、肝脾肿大为主要表现,发病年龄5岁以下;2外周血白细胞计数增高,持续单核细胞绝对值均>1.0×109/L,外周血易找到幼稚细胞;3骨髓细胞学提示骨髓增生明显活跃,原始细胞比例低于20%;4外周血血红蛋白F高于同年龄段正常值;5BCR/ABL融合基因阴性。结论:JMML多见于男性婴幼儿,临床表现与感染性疾病相似,易误诊,诊断过程中需注意外周血单核细胞比例及绝对值,血红蛋白F增高有助于诊断。

Objective：To analyze the clinical features and diagnosis of juvenile myelomonocytic leukaemia（JMML）.Method：Clinical data of 10 cases of JMML were retrospectively analyzed.Result：①The majority cases were boys,typical symptoms including fever,skin bleeding,enlarging abdomen and hepatosplenomegaly,and the age of onset was under 5years old.②The peripheral white blood cell count increased consistently,while monocyte count was persistently greater than 1.0×10^9/L and immature myeloid precursors were found easily in blood smears.③Bone marrow blasts were less than 20%.④Hemoglobin F was increased.⑤BCR/ABL gene rearrangement was absent.Conclusion：JMML is more common in male young children.It may be misdiagnosed as infectious diseases in which manifestations are very similar to JMML,increased monocytes and hemoglobin F are helpful for diagnosis of JMML.