von Willebrand factor-cleaving protease inhibitor in a patient with human immunodeficiency syndrome-associated thrombotic thrombocytopenic purpura.
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.
Sahud
Mervyn A
MA
Department of Hematology, San Francisco General Hospital, San Francisco, CA, USA. bloodres@pacbell.net
Claster
Susan
S
Liu
Lucy
L
Ero
Michael
M
Harris
Kathryn
K
Furlan
Miha
M
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*
D001323:Autoantibodies; D007074:Immunoglobulin G; D051722:ADAM Proteins; D008666:Metalloendopeptidases; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human