再生障碍性贫血发病机制研究进展

Advances in studies on the pathogenesis of aplastic anemia

再生障碍性贫血(AA)是以骨髓造血功能衰竭和高病死率为特征的血液系统"重症"之一,深入探索其发病机制进而开展靶向治疗,具有重要的临床价值和学术意义。传统观念认为AA为物理、化学或生物因素引起的造血组织"种子、虫子、土壤"异常、造血功能衰竭"综合征"。但部分骨髓移植植入失败患者其造血功能仍然能够自行恢复,同时临床中还发现大量患者经抗淋巴细胞球蛋白或抗胸腺细胞球蛋白(ATG/ALG)联合环孢素A治疗后获得了良好的疗效,说明了免疫机制异常在该病的发病中起到了重要的作用,因此,近年来AA已被国内外公认为一种涉及免疫机制异常所导致的自身免疫性疾病。文章就近年对该病发病机制的相关研究进行概述,以期为更好地阐述及完善再生障碍性贫血的病因提供帮助。

Aplastic anemia（AA） was one of the critical hematological diseases characterized by failure of bone marrow hematopoietic system and its high fatality rate. Further exploration on its pathogenesis in depth has important clinical value and great academic significance. Conventional concept considered AA as a syndrome of ＂seed/worms/soil＂ abnormity in hematopoietic tissue and failure of hematopoietic function caused by physical, chemical or biological factors. Yet the hematop0ietic function of part of SAA patients who experienced a failure of bone marrow implantation could still recover by itself. Meanwhile, in clinical cases, we found that a large number of AA patients obtained a good curative effect following the treatment of ATG/ALG combining CSA, which demonstrted that the abnormal immune mechanism played an important role in the pathogenesis of AA. Thus, in recent years, AA has been recognized as an autoimmune disease due to the abnormal immune mechanism. The present article is aimed to summarize the latest advances in the pathogenesis of AA, hoping to offer a help to elaborate and consummate the etiology ofaplastic anemia preferably.