加强对小儿骨髓增生异常综合征的认识

Improving the understanding of pediatric myelodysplastic syndrome

骨髓增生异常综合征是一组来源于造血干细胞的异质性的髓系恶性肿瘤，主要好发于老年人，儿童发病率很低，且与遗传性疾病高度相关。对于其发病机制的认识，近年来不断深入、细化，不仅发掘出多种与之相关的细胞遗传学及分子生物学改变，还针对其克隆演化的特点与疾病发生发展的关系，及协助预后判定方面有较系统的研究。现主要对以上机制加以总结，并结合儿童病例的特点进行阐述。

Myelodysplastic syndromes（MDS） represent a heterogeneous group of myeloid neoplasms initiated from stem cells and/or progenitor cells,which is primarily a disease of elderly. In childhood, MDS is uncommon. As for its mechanism, there has been a considerable improvement, involving cytogenetics, molecular biology. Besides, the relationship between hematological clonal evolution and initiation, development and prognosis of MDS, has also been reported systemically. These mechanisms are summarized and some characteristics of pediatric cases are reviewed.