TREATMENT OUTCOMES IN PATIENTS WITH ADULT THROMBOTIC THROMBOCYTOPENICPURPURA HEMOLYTIC-UREMIC SYNDROME

Background: Plasma treatment has improved the outcomes in adults with thrombotic thrombocytopenic purpura (TTP)-hemolytic uremic syndrome (H US). We reviewed our experience in treating unselected patients to det ermine the clinical outcomes and to evaluate the treatments given in a ddition to plasma. Methods: A chart review of all cases of TTP and HUS in adults treated at the Toronto (Ontario) Hospital, the largest trea tment center for adults with TTP-HUS in the province of Ontario, was c onducted. Results: Sixty-seven episodes of TTP-HUS in 52 consecutive a dult patients were treated during a 12-year period. Plasma was the pri mary form of therapy, and most patients received plasma exchange. A co mplete hematologic remission was achieved in 65 of 67 episodes; howeve r, two patients in remission were brain-dead. The time to complete rem ission varied from 3 to 58 days (median, 13 days). The death rate duri ng the acute illness was 8%. Long-term sequelae included relapses, per sisting renal impairment, hepatitis, and transfusion-associated acquir ed immunodeficiency syndrome. Relapses occurred in 21% of patients dur ing a median follow-up of 1.1 years (range, 0.1 to 18 years). Analyses of the treatment given in addition to plasma did not demonstrate a si gnificant benefit in terms of reducing the illness duration, mortality , or long-term sequelae. Conclusions: While most patients recovered fr om TTP-HUS, deaths still occurred and many patients suffered longterm complications. The role of the treatments given in addition to plasma is uncertain.