State-of-the-Art Review : Outcome of Thrombotic Thrombocytopenic Purpura and Hemolytic Uremic Syndrome in Japan

• Published in: Clinical and Applied Thrombosis/Hemostasis Vol. 5; no. 2; pp. 110 - 112
• Main Authors: Mori, Yoshitaka, Wada, Hideo, Tamaki, Shigehisa, Minami, Nobuyuki, Shiku, Hiroshi, Ihara, Toshiaki, Omine, Mituhiro, Kakisita, Eizo
• Format: Journal Article
• Language: English; Japanese
• Published: Thousand Oaks, CA SAGE Publications 01.04.1999; SAGE PUBLICATIONS, INC
• Subjects: Health risk assessment; Japan; TTP; Plasma exchange; Drug-induced HUS; Verotoxin-induced HUS
• ISSN: 1076-0296; 1938-2723
• DOI: 10.1177/107602969900500206

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.