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.

journal_name

Br J Haematol

authors

Sahud MA,Claster S,Liu L,Ero M,Harris K,Furlan M

doi

10.1046/j.0007-1048.2002.03349.x

subject

Has Abstract

pub_date

2002-03-01 00:00:00

pages

909-11

issue

4

eissn

0007-1048

issn

1365-2141

pii

3349

journal_volume

116

pub_type

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