title: The Japanese experience with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome.
abstract: A total of 290 Japanese patients with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) were analyzed with respect to ADAMTS-13 activity and its inhibitor. Twenty-one patients (17 families) had Upshaw-Schulman syndrome, and 12 patients (six families) had familial HUS of undetermined etiology. The number of patients with acquired HUS and TTP was 44 and 213, respectively. In acquired TTP, patients with severe deficiency of ADAMTS-13 activity secondary to the presence of an inhibitor were high responders to plasma exchange, but others were low responders to plasma exchange. The former patients were associated with "idiopathic" TTP, drugs, and pregnancy, and the latter patients with malignancy and stem cell transplantation. Patients with autoimmune disease-associated TTP fit into both groups.
authors.affiliation: Department of Blood Transfusion Medicine, Nara Medical University, Japan.
authors.forename: Masanori
authors.initials: M
authors.lastname: Matsumoto
authors.forename: Hideo
authors.initials: H
authors.lastname: Yagi
authors.forename: Hiromichi
authors.initials: H
authors.lastname: Ishizashi
authors.forename: Hideo
authors.initials: H
authors.lastname: Wada
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Fujimura
mesh_terms: D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; D000293:Adolescent; D000328:Adult; D000368:Aged; D044466:Asian People; D005260:Female; D006463:Hemolytic-Uremic Syndrome / Q000150:complications / Q000209:etiology / Q000235:genetics* / Q000503:physiopathology*; D006801:Humans; D007564:Japan / Q000453:epidemiology; D008297:Male; D008666:Metalloendopeptidases / Q000097:blood / Q000378:metabolism; D008875:Middle Aged; D011247:Pregnancy; D011697:Purpura, Thrombotic Thrombocytopenic / Q000150:complications / Q000209:etiology / Q000235:genetics* / Q000503:physiopathology*; D033581:Stem Cell Transplantation
chemical_list: D051722:ADAM Proteins; D008666:Metalloendopeptidases; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human