ACQUIRED VON-WILLEBRAND-DISEASE - CONCISE REVIEW OF OCCURRENCE, DIAGNOSIS, PATHOGENESIS, AND TREATMENT

Acquired von Willebrand disease (AvWD) is a rare complication of an au toimmune or neoplastic disease. It is associated mostly with a lymphoi d or plasma cell proliferative disorder. The clinical manifestations a re similar to congenital von Willebrand disease. Diagnosis is confirme d by the demonstration of decreased levels of factor VIII coagulant ac tivity (VIII:C), ristocetin cofactor activity (VWF:RCo), and von Wille brand factor (VWF) antigen (vWF:Ag). vWF multimer analysis usually rev eals a type II defect with decreased abundance of higher molecular wei ght vWF multimers. Various pathogenetic mechanisms have been described , including the development of anti-vWF antibodies and adsorption of v WF by tumor cells. Successful management approaches have included trea tment of the underlying disorder, infusion of high-dose gamma globulin , replacement therapy with factor VIII/vWF concentrates, intravenous i nfusion of desmopressin, and administration of corticosteroids. (C) 19 97 by Excerpta Medica, Inc.