儿童急性巨核细胞白血病临床生物学特征分析

目的探讨儿童急性巨核细胞白血病的临床及生物学特征。方法对11例AMKL患儿临床资料进行分析总结。结果实验室检查可见骨髓原幼巨核细胞比例增高>30%;骨髓纤维组织广泛增生;外周血或骨髓中检测到小巨核细胞;CD41、CD61表达明显增高;非特异性酯酶(NSE)、幼稚细胞糖元染色(PAS)、酸性磷酸酶(ACP)阳性率增高;电镜幼稚细胞血小板过氧化物酶(PPO)阳性;复杂染色体核型异常多见。结论AMKL占儿童AML7%~10%,多发生于婴幼儿,临床表现及生物学特征复杂,误诊率高,预后不良,应引起临床工作者的重视。

Analysis of clinical and biologic characteristics in pediatric acute megakaryoblastic leukemia

Objective To explore the clinical and biologic characteristics in Pediatric Acute Megakaryoblastic Leukemia (AMKL). Methods Analysis the clinical datas of 11 cases of AMKL. Results The ratio of megakaryoblast exeed 30% in bone marrow. Fibrous degeneration can be seen in bone marrow biopsy. Micromegakaryoblasts can be detected in periblood or bone marrow. The masculine ratios of CD41, CD61are higher than others. The masculine of nonspecific esterase(NSE), PAS, ACP can be seen in most of cases. Electron microscopy: platelet peroxidase was masculine. There were complicated karyotype abnormalities. Conclusion AMKL accounts for 7%~10% of pediatric acute myeloid leukemia. It occurs at a very early age in children. Most cases of AMKL are difficult to diagnose because of their complex clinical presentation and unusual bone marrow morphologic features. The prognosis is poor. We should pay more attention on it.