title: [Changes of ADAMTS13 activity and endothelial cell markers in TMA cases].
abstract: Severe deficiency of ADAMTS13 activity was recently found in patients with thrombotic thrombocytopenic purpura (TTP). The great advance associated with these basic and clinical studies on ADAMTS13 is the possible elucidation of the pathogenesis of TMA (thrombotic microangiopathy). However, the exact pathogenetic mechanism in TMA without severe deficiency of ADAMTS13 activity remains unknown due to heterogeneity of the disease. In this study, there were 7 patients with TTP, 7 with HUS, 3 with drug-induced HUS, 1 with VOD, and 1 with IVL out of 19 TMA patients with a moderate deficiency (6-70%) of ADAMTS13 activity. Five of the 7 TTP patients had a poor outcome. Plasma thrombomodulin and t-PA-PAI-1 complex levels in TMA patients with a moderate deficiency of ADAMTS13 activity were significantly higher than those in patients with a severe deficiency of ADAMTS13 activity. These data suggest that the etiology in these patients may be systemic vascular endothelial cell damage.
authors.affiliation: Mie Red Cross Blood Center, Japan.
authors.forename: Yoshitaka
authors.initials: Y
authors.lastname: Mori
authors.forename: Hideo
authors.initials: H
authors.lastname: Wada
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Shiku
authors.forename: Hideo
authors.initials: H
authors.lastname: Yagi
authors.forename: Masanori
authors.initials: M
authors.lastname: Matsumoto
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Fujimura
mesh_terms: D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; D000293:Adolescent; D000328:Adult; D000368:Aged; D015415:Biomarkers / Q000097:blood*; D005260:Female; D005337:Fibrin / Q000032:analysis; D006463:Hemolytic-Uremic Syndrome / Q000097:blood*; D006801:Humans; D008074:Lipoproteins / Q000097:blood; D008297:Male; D008666:Metalloendopeptidases / Q000097:blood*; D008875:Middle Aged; D010446:Peptide Fragments / Q000097:blood; D011498:Protein Precursors / Q000097:blood; D011516:Prothrombin; D011697:Purpura, Thrombotic Thrombocytopenic / Q000097:blood*; D013925:Thromboplastin / Q000032:analysis
chemical_list: D015415:Biomarkers; D008074:Lipoproteins; D010446:Peptide Fragments; D011498:Protein Precursors; C051928:lipoprotein-associated coagulation inhibitor; C465961:thrombus precursor protein, human; C029733:prothrombin fragment 1; C044183:prothrombin fragment 2; D011516:Prothrombin; D005337:Fibrin; D013925:Thromboplastin; D051722:ADAM Proteins; D008666:Metalloendopeptidases; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human