骨髓增生异常综合征的细胞凋亡分子机制(综述)

Progress in the study of abnormal molecular mechanism of myelodysplastic syndromes (A review)

骨髓增生异常综合征的基本病理特征是骨髓的无效造血。骨髓造血细胞的凋亡增加是导致无效造血的主要原因之一。目前认为 ,骨髓基质细胞分泌的细胞因子如肿瘤坏死因子 (TNF -α)、转化生长因子 β(TGF - β) ,Fas受体 /配体的高表达 ,凋亡调节基因的异常 ,如促凋亡基因 (Bax、Bad)与抗凋亡基因 (Bcl- 2、Bcl-X)的比值增高 ,及半胱氨酸 /天冬氨酸特异的蛋白酶 (caspases)的过度激活等 ,与病人的骨髓造血细胞凋亡增加有关。

Myelodysplastic syndromes are a group of clonal diseases pathologically characterized by ineffective hematopoiesis which is thought to be mainly due to abnormal apoptosis of hematopoietic cells. It is generally assumed that overexpression of some cytokines of stromal cells secrete (such as tumor necrosis factor α(TNF-α), transforming growth factor β(TGF-β), Fas receptor and Fas ligand), abnormality of apoptosis-regulating genes, (for instance, the ratio of pro-apoptotic genes such as Bax versus anti-apoptotic genes such as Bcl-2 increases), overactivation of cystein aspartate specific proteases (caspases) are correlated closely with increased apoptosis of patients' hematopoietic cells.