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Plasma exchange and vincristine in the treatment of hemolytic uremic syndrome/thrombotic thrombocytopenic purpura associated with bone marrow transplantation

✍ Scribed by Victor A. Silva; Debra Frei-Lahr; Randy A. Brown; Geoffrey P. Herzig


Publisher
John Wiley and Sons
Year
1991
Tongue
English
Weight
457 KB
Volume
6
Category
Article
ISSN
0733-2459

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✦ Synopsis


Hemolytic uremic syndrome/thrombotic thrornbocytopenic purpura (HUSITTP) is a rare and often fatal complication of bonc marrow transplantation (BMT). In this study. wc rcport eight such cases (seven allo and onc auto) treated with plasma exchanges (PE), vincristine (six paticnts), and discontinuation of cyclosporin A (in allo BMT). This complication occurred in 6.3% of 112 allogeneic BMT and in 0.7% of 146 autologous BMT. In seven patients, the BMT preparatory rcgimcns consisted of cyclophosphamide, etoposide, and total-body irradiation (TBI). Among the cight patients with HUSITTP. one allogeneic BMT patient with reversible renal failure, but without central nervous system (CNS) involvement, or systcmic mycotic infection. responded completely and is without evidence of disease for a pcriod of >3.5 years. Three patients showed hematologic improvement, and four did not respond. The median duration of survival was 17 days. Six of the scvcn deaths occurred in a setting of systemic infection, progressive renal failure, and worscning of graft-vcrsus-host reaction. In spite of hematologic improvemcnt to PE and vincristinc. BMT patients in whom HUSiTTP developed usually succumbcd to complications.