造血干细胞移植相关血栓性微血管病研究的新进展

Advances in hematopoietic stem cell transplant-associated thrombotic microangiopathy

造血干细胞移植（hematopoietic stem cell transplant,HSCT）是白血病与其他血液系统恶性肿瘤、再生障碍性贫血、某些先天性代谢与血红蛋白疾病的一种根治性措施,在我国已普遍开展。但HSCT可引起感染、移植物抗宿主病（graft-versushost disease,GVHD）以及出血与血栓性疾病等各种并发症,严重影响患者的预后。

Hematopoietic stem cell transplant-associated thrombotic microangiopathy is a severe complication with poor prognosis.Its pathogenesis is complex,and the manifestation is endothelial injury in the kidney and other organs.Recent studies demonstrate the role of complement activation in tissue damage.Tissue biopsy,monitor of blood pressure,de novo thrombocytopenia,de novo anemia and lactate dehydrogenase could be useful for early diagnosis.Therapeutic options remain limited.Withdrawal of offering agents such as calcineurin inhibitors is necessary.Eculizumab seems to be an effective drug with good outcomes.