【摘要】 目的分析幼年型粒单核细胞白血病(JMML)的临床特点。方法对19例JMML患儿的临床资料进行分析。结果 (1)JMML临床主要以发热、腹胀为主要症状,多见肝脾肿大。(2)外周血平均白细胞计数为37.29×109/L,血红蛋白平均80.7 g/L,血小板平均72.7×109/L,单核细胞绝对值均增高(>1.0×109/L)。(3)JMML均有HbF增高。(4)无Ph染色体。结论 JMML临床少见,依据目前诊断标准诊断不难,本病预后不良,目前唯一可以治愈本病的方法是异基因造血干细胞移植,而靶向治疗尚处于体外试验阶段。更多还原

【Abstract】 Objective To analysis the clinical features of Juvenile myelomonocytic leukaemia(JMML).Methods Clinical data of 19 cases of JMML were analyzed.Results From 19 JMML patients we found:(1)The main symptoms were fever,abdominal distention,hepatosplenomegaly.(2)The medium WBC count,hemoglobin and platelet in peripheral blood were 37.29×109/L,80.7 g/L and 72.7×109/L respectively,absolute value of monocyte counts of all the cases were >1.0×109/L.(3)Fetal hemoglobin increased.(4)Absence of Philadelphia chromosome.Conclusions JMML is rare in clinic with unfavorable prognosis,and it is not difficult to diagnose according to present diagnostic criteria.Currently,the only curable method is allogeneic bone marrow transplantation,while target therapy of the gene is still in the extro-experiment stage.更多还原