移植相关性血栓性微血管病临床研究

Clinical Study of Transplant-Associated Thrombotic Microangiopathy

造血干细胞移植(Hematopoietic stem cell transplant HSCT)相关血栓性微血管病(Hematopoietic stem cell transplant-associated thrombotic microangiopathy TA-TMA)是公认的移植后出现的严重并发症。其特征是微血管病性溶血性贫血、原因不明的血小板减少症、乳酸脱氢酶(Lactate dehydrogenase, LDH)升高和内皮损伤所致相关器官衰竭为特征,如高血压、慢性肾病、肺动脉高压、胃肠道或中枢神经系统疾病。TA-TMA的临床表现与血栓性血小板减少性紫癜(thrombotic thrombocytopenic purpura, TTP)和非典型溶血性尿毒症综合征(atypical haemolytic uraemic syndrome, aHUS)相似,后两者以微血管病性溶血性贫血、血小板消耗、微循环纤维蛋白沉积以及最终的终末器官损伤为特征。由于该病起病隐匿,进展迅速,对其认识不足,经常造成漏诊和误诊。我们应该及早发现,及早干预,降低患者死亡风险。

Hematopoietic stem cell transplant (HSCT)-associated thrombotic microangiopathy TA-TMA is rec-ognized as a serious complication after transplantation. It is characterized by microangiopathic he-molytic anemia, unexplained thrombocytopenia, elevated lactate dehydrogenase (LDH), and related organ failure due to endothelial damage, such as hypertension, chronic kidney disease, pulmonary hypertension, gastrointestinal or central nervous system diseases. The clinical manifestations of TA-TMA were correlated with thrombotic thrombocytopenic purpura (TTP) and atypical haemolytic uraemic syndrome (aHUS), characterized by microangiopathic hemolytic anemia, platelet depletion, microcirculating fibrin deposition, and eventual end-organ damage. Due to the hidden onset, rapid progress and insufficient understanding of the disease, it often leads to missed diagnosis and misdiagnosis. We should make early detection and intervention to reduce the risk of death.