Background Thrombotic thrombocytopenic purpura is a potentiallyfatal disease characterized by widespread platelet thrombi inthe microcirculation. In the normal circulation, von Willebrandfactor is cleaved by a plasma protease. We explored the hypothesisthat a deficiency of this protease predisposes patients withthrombotic thrombocytopenic purpura to platelet thrombosis.
Methods We studied the activity of von Willebrand factor–cleavingprotease and sought inhibitors of this protease in plasma frompatients with acute thrombotic thrombocytopenic purpura, patientswith other diseases, and normal control subjects. We also investigatedthe effect of shear stress on the ristocetin cofactor activityof purified von Willebrand factor in the cryosupernatant fractionof the plasma samples.
Results Thirty-nine samples of plasma from 37 patients withacute thrombotic thrombocytopenic purpura had severe deficiencyof von Willebrand factor–cleaving protease. No deficiencywas detected in 16 samples of plasma from patients with thromboticthrombocytopenic purpura in remission or in 74 plasma samplesfrom normal subjects, randomly selected hospitalized patientsor outpatients, or patients with hemolysis, thrombocytopenia,or thrombosis from other causes. Inhibitory activity againstthe protease was detected in 26 of the 39 plasma samples (67percent) obtained during the acute phase of the disease. Theinhibitors were IgG antibodies. Shear stress increased the ristocetincofactor activity of von Willebrand factor in the cryosupernatantof plasma samples obtained during the acute phase, but decreasedthe activity in cryosupernatant of plasma from normal subjects.
Conclusions Inhibitory antibodies against von Willebrand factor–cleavingprotease occur in patients with acute thrombotic thrombocytopenicpurpura. A deficiency of this protease is likely to have a criticalrole in the pathogenesis of platelet thrombosis in this disease.
Source Information
From the Division of Hematology, Montefiore Medical Center and Albert Einstein College of Medicine, Bronx, N.Y. (H.-M.T.); and the Hemophilia and Thrombosis Center and Sylvester Cancer Center, University of Miami and Veterans Affairs Medical Center, Miami (E.C.-Y.L.).
Address reprint requests to Dr. Tsai at Montefiore Medical Center, Division of Hematology, 111 E. 210th St., Bronx, NY 10467.
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