Acquired von Willebrand's disease due to aberrant expression of platelet glycoprotein Ib by marginal zone lymphoma cells |
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| Authors: | AYALEW,TEFFERI ,CURTIS A.,HANSON ,PAUL J.,KURTIN ,JERRY A.,KATZMANN ,ROBERT J.,DALTON & WILLIAM L.,NICHOLS |
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| Affiliation: | Division of Hematology and Internal Medicine; Division of Hematopathology, Mayo Clinic, Rochester;, Department of Hematology and Oncology, Duluth Clinic, Duluth, Minnesota, U.S.A. |
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| Abstract: | A 69-year-old woman presented with splenic marginal zone lymphoma associated with acquired von Willebrand's disease (AVWD). Laboratory abnormalities included markedly decreased plasma levels of factor VIII coagulant (C) activity (VIII:C 28%), von Willebrand's factor (VWF) antigen (Ag) (vWF:Ag < 6%), and VWF ristocetin cofactor (RCo) activity (VWF:RCo, < 12%). VWF multimer analysis revealed a severe type II defect. Treatment with cryoprecipitate, high-dose gamma globulin or desmopressin given intravenously was unsuccessful. Clinical bleeding and coagulation abnormalities showed transient improvement after replacement therapy with Humate-P concentrate. The coagulation abnormalities improved partially after splenectomy and completely after subsequent chemotherapy. The neoplastic lymphocytes in the blood and spleen strongly expressed platelet glycoprotein Ib (CD42) and VWF but not other platelet-associated antigens. |
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| Keywords: | acquired von Willebrand's disease glycoprotein Ib lymphoma |
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