儿童急性巨核细胞白血病9例分析

目的分析儿童急性巨核细胞白血病患者的临床特征、免疫表型、细胞遗传学特征,为临床诊断及进一步治疗提供相关依据。方法对我院确诊的9例急性巨核细胞白血病患者的临床表现、实验室检查进行分析。结果9例发病年龄均小于3岁,男∶女为7∶2,89%患者急性起病,89%患者因出血就诊。肝大患者占78%,所有患者均出现重度血小板减少,中度贫血患者占89%。骨髓幼稚巨核细胞均大于20%。所有患者肿瘤细胞均表达CD41抗原。3例患者伴有染色体复杂核型异常。本组患者均失访。结论1.本病临床是以出血、肝脏肿大为主要表现,其发病年龄小,急性起病、男性较多见。2.常伴血小板重度减少,免疫标记CD41细胞是急性巨核细胞白血病快捷的诊断方法,本病可伴有复杂核型。3.预后差。

Clinical features of acute megakaryocytic leukemia in 9 children cases

Objective To assess the clinical features, immunophenotypic, and cytogenetic characteristics of Acute Megakaryocytic Leukemia(AMKL), to indicate the diagnose and treatment in clinic. MethodsAnalysis clinical and laboratory data of 9 children with AMKL in BCH Hematology center. ResultsAll patients were younger than 3 year-old, the ratio of male and female was 7∶2, acute onset in 89%, hepatomegaly was in 78%, all patients followed with severe thrombocytopenia, and 89% with moderate anemia. Bone marrow megakaryoblasts were more than 20%. All were positive in CD41. Chomosome complicated aberrations in 3 cases. Conclusions1. AMKL is frequent in children younger than 3 years of age, it usually have acute onset. Male is frequent, hemorrhage and hepatomagely were main clinical features. 2. Patients present with severe thrombocytopenia. The effective diagnosis of AMKL is the expression of platelet specific CD41 antigen. 3. AMKL is a bad prognosis disease.