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Haemolytic uraemic syndrome following bone marrow transplantation. Case report and review of the literature
Authors:Verburgh  C A; Vermeij  C G; Zijlmans  J M; van Veen  S; van Es  L A
Institution:1Department of Nephrology, University Hospital Leiden Leiden, The Netherlands 2Department of Haematology, University Hospital Leiden Leiden, The Netherlands
Abstract:Thrombotic microangiopathy (TMA) can be a late complicationof bone marrow transplantation (BMT). A patient is describedin whom the haemolytic uraemic syndrome developed 10 monthsafter BMT and who died of E. coli sepsis while on maintenancehaemodialysis. The literature is reviewed, regarding clinicalpresentation, incidence, pathogenesis and therapy. TMA can beobserved, after an interval of 3–12 months, in about 6–26%of patients following BMT. Reported cases vary considerablyin clinical severity, from mild presentations to severe TMAwith high mortality rates despite intensive therapy. Importantpathogenetic roles are ascribed to the conditioning total bodyirradiation and the use of cyclosporin A, but other factorsmay be involved as well. Next to supportive therapy, plasmaexchange and the use of ACE inhibitors may be of value in treatingBMT-associated TMA.
Keywords:bone marrow transplantation  cyclosporin A  haemolytic uraemic syndrome  thrombotic microangiopathy  thrombotic thrombocytopenic purpura  total body irradiation
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