Acquired von Willebrand disease.
Acquired von Willebrand disease (AvWD) is an acquired bleeding disorder which may suddenly become manifest in individuals, usually in the absence of a personal or family history of bleedings and frequently in association with monoclonal gammopathies, lymphoproliferative, myeloproliferative and autoimmune disorders. In a minority of the cases AvWD may develop in association with drugs or solid tumours. Pathogenetic mechanisms involve autoantibodies directed against von Willebrand factor (vWF) resulting in a rapid clearance of vWF from the circulation and/or inactivation of plasma vWF; absorption or adsorption of plasma vWF to malignant cells; drug-induced or cell-mediated proteolysis of plasma vWF; acquired decrease in synthesis of vWF and/or release of vWF from storage sites; or precipitation of plasma vWF. Treatment options include--whenever possible--treatment of the underlying disorder or symptomatic treatment aimed at replacing the loss of vWF by either infusion of vWF-rich concentrates or administration of desmopressin (DDAVP). In selected cases with anti-vWF antibodies, administration of high-dose intravenous gammaglobulin, plasma exchange or extracorporeal immunoadsorption may be successful.
van Genderen
P J
PJ
Department of Haematology, University Hospital Dijkzigt, Rotterdam, The Netherlands.
Michiels
J J
JJ
D002648:Child; D005260:Female; D006801:Humans; D008297:Male; D014842:von Willebrand Diseases / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D014841:von Willebrand Factor / Q000276:immunology / Q000378:metabolism
D014841:von Willebrand Factor