Acquired von Willebrand disease: Concise review of occurrence, diagnosis, pathogenesis, and treatment

Ayalew Tefferi, William L. Nichols

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Acquired von Willebrand disease (AvWD) is a rare complication of an autoimmune or neoplastic disease. It is associated mostly with a lymphoid or plasma cell proliferative disorder. The clinical manifestations are similar to congenital von Willebrand disease. Diagnosis is confirmed by the demonstration of decreased levels of factor VIII coagulant activity (VIII:C), ristocetin cofactor activity (vWF:RCo), and von Willebrand factor (vWF) antigen (vWF:Ag). vWF multimer analysis usually reveals a type II defect with decreased abundance of higher molecular weight vWF multimers. Various pathogenetic mechanisms have been described, including the development of anti-vWF antibodies and adsorption of vWF by tumor cells. Successful management approaches have included treatment of the underlying disorder, infusion of high-dose gamma globulin, replacement therapy with factor VIII/vWF concentrates, intravenous infusion of desmopressin, and administration of corticosteroids.

Original languageEnglish (US)
Pages (from-to)536-540
Number of pages5
JournalAmerican Journal of Medicine
Issue number6
StatePublished - Dec 1997


ASJC Scopus subject areas

  • Nursing(all)

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