Abstract
We examined 159 patients with thrombotic throm bocytopenic purpura and hemolytic uremic syndrome in Japan. The subjects were divided in three groups; 90 patients with thrombotic thrombocytopenic purpura, 51 patients with vero toxin-induced hemolytic uremic syndrome, and 18 patients with drug-induced hemolytic uremic syndrome. Eighty-two percent of the patients with thrombotic thrombocytopenic pur pura had associated neurological disorders and 78% of drug- induced hemolytic uremic syndrome associated with pulmo nary edema. Renal insufficiency was noted in the 69% cases with both hemolytic uremic syndrome groups. Seventeen pa tients with thrombotic thrombocytopenic purpura had systemic lupus erythematosus and 6 were pregnant. Autoantibody were positive in 53% of thrombotic thrombocytopenic purpura. Sev enty-seven percent of patients with thrombotic thrombocytope nic purpura received plasma exchange at 4,000 mL/day three times a week, 71 % antithrombotic agents, and 78% steroid administration, respectively. However, 27% of the patients with hemolytic uremic syndrome were treated by hemodialysis in addition to antithrombotic agents. When drug-induced hemo lytic uremic syndrome was diagnosed, the drug was immedi ately discontinued and the patients were treated with antiplate let agents. Seventy-four percent of the patients with thrombotic thrombocytopenic purpura were alive at 26 weeks compared with 95% of those with hemolytic uremic syndrome. As throm botic thrombocytopenic purpura had a high mortality rate in Japan, we should carry out early diagnosis and early treatment.