Verburgh C A, Vermeij C G, Zijlmans J M, van Veen S, van Es L A
Department of Nephrology, University Hospital Leiden, Leiden, The Netherlands.
Nephrol Dial Transplant. 1996 Jul;11(7):1332-7.
Thrombotic microangiopathy (TMA) can be a late complication of bone marrow transplantation (BMT). A patient is described in whom the haemolytic uraemic syndrome developed 10 months after BMT and who died of E. coli sepsis while on maintenance haemodialysis. The literature is reviewed, regarding clinical presentation, incidence, pathogenesis and therapy. TMA can be observed, after an interval of 3-12 months, in about 6-26% of patients following BMT. Reported cases vary considerably in clinical severity, from mild presentations to severe TMA with high mortality rates despite intensive therapy. Important pathogenetic roles are ascribed to the conditioning total body irradiation and the use of cyclosporin A, but other factors may be involved as well. Next to supportive therapy, plasma exchange and the use of ACE inhibitors may be of value in treating BMT-associated TMA.
血栓性微血管病（TMA）可能是骨髓移植（BMT）的晚期并发症。本文描述了一名患者，其在骨髓移植10个月后发生溶血性尿毒症综合征，并在维持性血液透析期间死于大肠杆菌败血症。本文对有关临床表现、发病率、发病机制和治疗的文献进行了综述。骨髓移植后3 - 12个月，约6% - 26%的患者可观察到TMA。报告的病例在临床严重程度上差异很大，从轻度表现到严重的TMA，尽管进行了强化治疗，但死亡率仍很高。全身照射预处理和使用环孢素A被认为在发病机制中起重要作用，但其他因素可能也有影响。除支持性治疗外，血浆置换和使用血管紧张素转换酶抑制剂可能对治疗与骨髓移植相关的TMA有价值。
