HEMOLYTIC-UREMIC SYNDROME FOLLOWING BONE-MARROW TRANSPLANTATION - CASE-REPORT AND REVIEW OF THE LITERATURE

Thrombotic microangiopathy (TMA) can be a late complication of bone ma rrow transplantation (BMT). A patient is described in whom the haemoly tic uraemic syndrome developed 10 months after BMT and who died of E. coli sepsis while on maintenance haemodialysis. The literature is revi ewed, regarding clinical presentation, incidence, pathogenesis and the rapy. TMA can be observed, after an interval of 3-12 months, in about 6-26% of patients following BMT. Reported cases vary considerably in c linical severity, from mild presentations to severe TMA with high mort ality rates despite intensive therapy. Important pathogenetic roles ar e ascribed to the conditioning total body irradiation and the we of cy closporin A, but other factors may be involved as well. Next to suppor tive therapy, plasma exchange and the use of ACE inhibitors may be of value in treating BMT-associated TMA.