title: von Willebrand factor-cleaving protease inhibitor in a patient with human immunodeficiency syndrome-associated thrombotic thrombocytopenic purpura.
abstract: Antibodies that inhibit von Willebrand Factor (VWF)-cleaving protease activity occur in patients with acute thrombotic thrombocytopenic purpura (TTP) and often persist in the chronic phase. A deficiency of this protease is likely to be responsible for the generation of ultrahigh VWF multimers and influence the formation of intra-arterial platelet aggregates that result in microangiopathic haemolytic anaemia, thrombocytopenia and end in organ failure. This report demonstrates complete deficiency of VWF-cleaving protease and the presence of a concentration-dependent IgG1 inhibitor in the plasma of a patient with acquired immunodeficiency syndrome (AIDS). These data may contribute to understanding the pathophysiology of human immunodeficiency syndrome (HIV)-related TTP.
authors.affiliation: Department of Hematology, San Francisco General Hospital, San Francisco, CA, USA. bloodres@pacbell.net
authors.forename: Mervyn A
authors.initials: MA
authors.lastname: Sahud
authors.forename: Susan
authors.initials: S
authors.lastname: Claster
authors.forename: Lucy
authors.initials: L
authors.lastname: Liu
authors.forename: Michael
authors.initials: M
authors.lastname: Ero
authors.forename: Kathryn
authors.initials: K
authors.lastname: Harris
authors.forename: Miha
authors.initials: M
authors.lastname: Furlan
mesh_terms: D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; D000163:Acquired Immunodeficiency Syndrome / Q000150:complications / Q000276:immunology*; D001323:Autoantibodies / Q000097:blood*; D004796:Clinical Enzyme Tests; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D008297:Male; D008666:Metalloendopeptidases / Q000032:analysis / Q000276:immunology*; D008875:Middle Aged; D011697:Purpura, Thrombotic Thrombocytopenic / Q000150:complications / Q000276:immunology*
chemical_list: D001323:Autoantibodies; D007074:Immunoglobulin G; D051722:ADAM Proteins; D008666:Metalloendopeptidases; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human