移植相关血栓性微血管病的研究进展

移植相关血栓性微血管病(TA-TMA),是一类以微血管病性溶血性贫血、肾功能不全和神经系统异常为主要特征的造血干细胞移植(HSCT)并发症.TA TMA发病机制可能与移植预处理、病原微生物感染、钙调磷酸酶及雷帕霉素靶蛋白(mTOR)抑制剂、移植物抗宿主病(GVHD)、细胞因子、补体及中性粒细胞胞外陷阱(NET)等多种因素相关.临床对TA-TMA的诊断标准,目前仍局限于有创性较大的组织病理学诊断,缺乏有创性较小的特异性生物标志物诊断手段.目前,治疗性血浆置换、GVHD预防药物的调整、去纤苷、利妥昔单抗及依库珠单抗等,可能是治疗TA-TMA的有效手段.随着对TA-TMA发病机制研究的深入,制定其标准诊断和治疗方案正受到国内外广泛关注.笔者拟就TA-TMA的研究进展综述如下.

Research progress of transplant-associated thrombotic microangiopathy

Transplant-associated thrombotic microangiopathy (TA-TMA is a significant complication of hematopoietic stem cell transplantation (HSCT) manifesting mainly as microangiopathic hemolytic anemia,renal insufficiency and neurological abnormalities.The pathogenesis of TA-TMA is related with HSCT conditioning regimens,infections,calcineurin and mammalian target of rapamycin (mTOR) inhibitors,graft-versus host disease (GVHD),cytokines,complements and neutrophil extracellular trap (NET).The diagnostic criteria of TA-TMA are still confined to clinicopathological diagnosis with great invasive and lack of specific biomarker.Therapeutic plasma exchange,manipulation of GVHD prophylaxis,defibrotide,rituximab and eculizumab may be helpful in the treatment of TA-TMA.With the further research of the pathogenesis of TA-TMA,its standard diagnosis and treatment protocols are of international concern.And this review will focus on the research progress of TA-TMA.