骨髓增生异常综合征铁超载机制的研究进展

Progress on the mechanism of iron overload in myelodysplastic syndrom

骨髓增生异常综合征（myelodysplastic syndromes，MDS）是一组起源于造血干/祖细胞的获得性髓系克隆性疾病。研究表明，在MDS诊断及支持治疗的过程中患者出现不同程度的铁代谢异常。体内铁超负荷可破坏机体铁平衡，过量的游离铁沉积在血浆及组织中，引起机体主要脏器如心脏、肝脏及内分泌腺体并发症，促进MDS向急性髓系白血病转化，并可导致病死率增高。祛铁治疗可以改善患者预后，延长其总生存期及无白血病生存期。该文对MDS铁超载发生机制进行综述。

Myelodysplastic syndrome （MDS） is a group of origin of hematopoietic stem/progenitor cells of myeloid clonal disorders. Studies have shown that MDS patients are with varying degrees of abnormal iron metabolism in the course of diagnosis and supportive care. Iron overload can damage the body＇s iron balance. Excess free iron can sink in plasma and tissue, especially in the body＇s major organs such as heart, liver and endocrine gland,resulting in structural damage and organ dysfunction, accelerating transformation to acute myeloid leukemia, and increasing the death rate. Iron chelation can improve the prognosis of patients,prolong overall survival and leukemia-free survival. The mechanism of iron overload in MDS research will be described in this paper.