title: Outcome of thrombotic thrombocytopenic purpura and hemolytic uremic syndrome in Japan.
abstract: We examined 159 patients with thrombotic thrombocytopenic purpura and hemolytic uremic syndrome in Japan. The subjects were divided in three groups; 90 patients with thrombotic thrombocytopenic purpura, 51 patients with verotoxin-induced hemolytic uremic syndrome, and 18 patients with drug-induced hemolytic uremic syndrome. Eighty-two percent of the patients with thrombotic thrombocytopenic purpura had associated neurological disorders and 78% of drug-induced hemolytic uremic syndrome associated with pulmonary edema. Renal insufficiency was noted in the 69% cases with both hemolytic uremic syndrome groups. Seventeen patients with thrombotic thrombocytopenic purpura had systemic lupus erythematosus and 6 were pregnant. Autoantibody were positive in 53% of thrombotic thrombocytopenic purpura. Seventy-seven percent of patients with thrombotic thrombocytopenic 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 hemolytic uremic syndrome was diagnosed, the drug was immediately discontinued and the patients were treated with antiplatelet 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 thrombotic thrombocytopenic purpura had a high mortality rate in Japan, we should carry out early diagnosis and early treatment.
authors.affiliation: Second Department of Internal Medicine, Mie University School of Medicine, Tsu-city, Japan.
authors.forename: Y
authors.initials: Y
authors.lastname: Mori
authors.forename: H
authors.initials: H
authors.lastname: Wada
authors.forename: S
authors.initials: S
authors.lastname: Tamaki
authors.forename: N
authors.initials: N
authors.lastname: Minami
authors.forename: H
authors.initials: H
authors.lastname: Shiku
authors.forename: T
authors.initials: T
authors.lastname: Ihara
authors.forename: M
authors.initials: M
authors.lastname: Omine
authors.forename: E
authors.initials: E
authors.lastname: Kakisita
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001241:Aspirin / Q000008:administration & dosage; D001427:Bacterial Toxins; D002648:Child; D002675:Child, Preschool; D002986:Clinical Trials as Topic; D018572:Disease-Free Survival; D005260:Female; D005343:Fibrinolytic Agents / Q000008:administration & dosage; D006463:Hemolytic-Uremic Syndrome / Q000139:chemically induced / Q000188:drug therapy*; D006493:Heparin / Q000008:administration & dosage; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007564:Japan / Q000453:epidemiology; D008297:Male; D008775:Methylprednisolone / Q000008:administration & dosage; D008875:Middle Aged; D016685:Mitomycin / Q000008:administration & dosage; D010951:Plasma Exchange; D010975:Platelet Aggregation Inhibitors / Q000627:therapeutic use; D011247:Pregnancy; D011697:Purpura, Thrombotic Thrombocytopenic / Q000188:drug therapy*; D012008:Recurrence; D006435:Renal Dialysis; D022622:Shiga Toxin 1; D013256:Steroids / Q000008:administration & dosage; D015996:Survival Rate; D016896:Treatment Outcome
chemical_list: D001427:Bacterial Toxins; D005343:Fibrinolytic Agents; D010975:Platelet Aggregation Inhibitors; D022622:Shiga Toxin 1; D013256:Steroids; D016685:Mitomycin; D006493:Heparin; D001241:Aspirin; D008775:Methylprednisolone