von Willebrand factor-cleaving protease inhibitor in a patient with human immunodeficiency syndrome-associated thrombotic thrombocytopenic purpura

Br J Haematol. 2002 Mar;116(4):909-11. doi: 10.1046/j.0007-1048.2002.03349.x.


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.

Publication types

  • Case Reports

MeSH terms

  • ADAM Proteins
  • ADAMTS13 Protein
  • Acquired Immunodeficiency Syndrome / complications
  • Acquired Immunodeficiency Syndrome / immunology*
  • Autoantibodies / blood*
  • Clinical Enzyme Tests
  • Humans
  • Immunoglobulin G / blood*
  • Male
  • Metalloendopeptidases / analysis
  • Metalloendopeptidases / immunology*
  • Middle Aged
  • Purpura, Thrombotic Thrombocytopenic / complications
  • Purpura, Thrombotic Thrombocytopenic / immunology*


  • Autoantibodies
  • Immunoglobulin G
  • ADAM Proteins
  • Metalloendopeptidases
  • ADAMTS13 Protein
  • ADAMTS13 protein, human