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Therapeutic plasma exchange does not appear to be effective in the management of thrombotic thrombocytopenic purpura/hemolytic uremic syndrome following bone marrow transplantation. Bone Marrow Transplant 1995 Aug;16(2):271-5

Date

08/01/1995

Pubmed ID

7581147

Scopus ID

2-s2.0-0029150064 (requires institutional sign-in at Scopus site)   99 Citations

Abstract

Recognition of thrombotic thrombocytopenic purpura (TTP)/hemolytic uremic syndrome (HUS) following BMT has increased in recent years. The pathogenesis and etiology may be related to endothelial cell damage secondary to irradiation and/or CsA. Optimal management of this condition remains unclear. Due to similarity between this syndrome and classical TTP, patients with TTP/HUS following BMT are commonly treated with therapeutic plasma exchange (TPE). We describe our experience with 9 such patients who were treated with TPE (8 cases) and immunoadsorption with a Staphylococcal Protein A column (1 case). The exchanges were done with fresh frozen plasma and/or cryoprecipitate-depleted frozen plasma. Out of 8 patients treated with TPE, 6 died within 2 months of TPE due to secondary infections, metabolic disturbances and progression of TTP/HUS. Of these 6 patients, 5 had no hematological response, while 1 had hematological improvement. Two patients are alive 4 and 3 years later, however, they had shown only minimal hematological response at the end of 28 and 20 TPE, respectively. Their renal function remains stable but severely reduced. The ninth patient who received Staphylococcal Protein A column treatment died within 5 days of treatment without hematological improvement. Thus, in contrast to its effectiveness in classical TTP, TPE does not appear to be as effective in the management of well established TTP/HUS following BMT.

Author List

Sarode R, McFarland JG, Flomenberg N, Casper JT, Cohen EP, Drobyski WR, Ash RC, Horowitz MM, Camitta B, Lawton C

Authors

Bruce m. Camitta Professor in the Pediatrics department at Medical College of Wisconsin
James Casper MD Emeritus Professor in the Pediatrics department at Medical College of Wisconsin
William R. Drobyski MD Professor in the Medicine department at Medical College of Wisconsin
Mary M. Horowitz MS, MD Adjunct Professor in the Medicine department at Medical College of Wisconsin
Colleen A. Lawton MD Emeritus Professor in the Radiation Oncology department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Adolescent
Adult
Bone Marrow Transplantation
Female
Hemolytic-Uremic Syndrome
Hepatic Veno-Occlusive Disease
Humans
Male
Middle Aged
Plasma Exchange
Purpura, Thrombotic Thrombocytopenic