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

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Abstract
Summary. 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.