Thrombotic thrombocytopenic purpura: The israeli experience

Since 1985, 13 patients with thrombotic thrombocytopenic purpura (TTP) have been treated at the Hadassah Hospital. 1 patient died during the acute episode and eight had one or more relapses. Using a scoring system, developed in a previous report (Am J Med 1987; 83:437) to assess the severity of each episode, we found that 9 patients presented with severe TTP (score of 5-7) and four had milder symptoms (score 2-4). As reported previously, relapses were usually manifested by a milder clinical course, however 1 patient died during a relapse. The treatment for the initial TTP episode and the relapses consisted of steroids, platelet inhibitor drugs, vincristine and frequent plasma transfusions or exchanges and varied according to the severity of the disease. 4 patients underwent splenectomy. Two who failed to respond to conventional therapy underwent splenectomy and recovered following additional plasma therapy. The other two underwent splenectomy while in remission, in order to prevent frequent relapses. A significant favourable change in the course of their disease was observed.