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Pseudo-Bernard-Soulier syndrome: thrombocytopenia caused by autoantibody to platelet glycoprotein Ib

DV Devine, MS Currie, WF Rosse and CS Greenberg

The Bernard-Soulier syndrome is an inherited bleeding disorder that is due to a deficiency in platelet glycoprotein Ib. Bernard-Soulier platelets fail to agglutinate in response to ristocetin despite normal levels of factor VIII:von Willebrand factor. We report a patient who developed severe refractory thrombocytopenia postsurgically while receiving procainamide therapy. Thrombocytopenia was immune mediated since the patient's platelets bore high levels of antiplatelet antibody. Radioimmunoprecipitation studies demonstrated that the autoantibodies had specificity for platelet glycoproteins Ib and V as well as platelet HLA. The patient's plasma as well as purified immunoglobulin G completely inhibited the ristocetin-induced aggregation of normal platelets but did not inhibit adenosine diphosphate-induced aggregation. The laboratory studies revealed that this patient suffered from antibody-mediated thrombocytopenia with unusual characteristics that we have called pseudo-Bernard-Soulier syndrome.

Volume 70, Issue 2, pp. 428-431, 08/01/1987
Copyright © 1987 by The American Society of Hematology


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J. A. Lopez, R. K. Andrews, V. Afshar-Kharghan, and M. C. Berndt
Bernard-Soulier Syndrome
Blood, June 15, 1998; 91(12): 4397 - 4418.
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  Copyright © 1987 by American Society of Hematology         Online ISSN: 1528-0020