小儿骨髓增生异常综合征17例临床分析

Clinical analysis of myelodysplastic syndrome in 17 children

为进一步探讨小儿骨髓增生异常综合征(MDS)的临床特点,对近5年诊断的17例小儿MDS进行分析,其中RA1例,RAEB 7例,RAEB-t 5例,CMML 4例,外周血均有一系或一系以上血细胞减少,骨髓各细胞系统均有不同程度病态造血。本文对小儿低增生型MDS、CMML的分型诊断进行讨论,并提出淋巴系统病态造血可能是小儿MDS的特点之一。

To explore the clinical characteristics of childhood myelodysplastic syndrome (MDS), clinical data were analyzed in 17 children with MDS, who were diagnosed in recent five years. It was classified that one case was as refractory anemia (RA), 7 cases as refractory anemia with excess blasts (RAEB), 5 cases as RAEB-transformation (RAEB-t) and 4 cases as chronic myelo-monocytic leukemia (CMML), respectively. Cytopenia in at least one cell line was observed in all patients' peripheral blood, and ineffective haemopoiesis at different extent was further confirmed in different hemopoietic lines of their bone marrow. It is suggested that ineffective haemopoiesis of the lymphoid cell line may be one of the features in childhood MDS.