ALPHA(1)-ANTITRYPSIN THERAPY IN A CASE OF THROMBOTIC THROMBOCYTOPENICPURPURA

In thrombotic thrombocytopenic purpura (TTP), activated leucocytes rel ease elastase which large von Willebrand factor (vWF) endothelium. We investigated the effect of an inhibitor of leucocyte elastase (alpha(1 )-antitrypsin) on circulating vWF and platelet count in a patient with chronic relapsing TTP. alpha(1)-antitrypsin consistently normalised v WF multimeric composition but failed to Increase platelet count and in duce remission. Plasma cryosupernatant, which never normalised VWF, al ways induced laboratory and clinical remission within 96 hours. In chr onic relapsing TTP, unusually large VWF multimers are not the main cau se of intravascular platelet aggregation.