Response to plasma exchange and steroids as combined therapy for patients with thrombotic thrombocytopenic purpura

We describe our experience in the management of 11 consecutive patients wit h thrombotic thrombocytopenic purpura (TTP) treated with a combined therapy of plasma exchange (PE) and steroids. Nine patients (82%) achieved complet e remission (CR) after a median of 6 rounds of PE (range 2-22). There were 3 early relapses managed in the same way as the initial episode. One patien t relapsed 23 months after diagnosis achieving CR with standard therapy; an other patient suffered several relapses, and splenectomy was performed afte r the last one. Three patients died, 2 of them with resistant disease 9 and 38 days after diagnosis, and the remaining one died due to AIDS-related co mplications while he was in CR. Eight patients are alive in CR with a media n followup of 38 months (range 8-74). The combination of PE and steroids is a well-tolerated and effective treatment of TTP, but improvements in thera py are needed to manage refractory patients.