Acquired von Willebrand syndrome: features and management

Abstract

Acquired von Willebrand syndrome (AvWS) is not a well-known bleeding disorder among clinicians and is associated with various underlying diseases. The clinical manifestations are similar to congenital von Willebrand disease. Diagnosis is confirmed mainly by a decrease of ristocetin cofactor activity (vWF:RCo) and/or collagen binding activity (vWF:CBA) and by vWF multimeric analysis, usually with a selective loss of large multimers. Plasma von Willebrand factor propeptide (vWF:AgII) is a good marker of vWF synthesis. Various pathogenic mechanisms have been proposed, including development of autoantibodies to the von Willebrand factor (vWF), adsorption of vWF onto tumor cells or activated platelets, increase of vWF proteolysis, and mechanical destruction of vWF under high shear stress. Treatment of the underlying disorder may resolve AvWS. Desmopressin (DDAVP) is a first-line therapeutic option. Factor VIII/vWF concentrates and high-dose immunoglobulin infusions are reserved for patients unresponsive to DDAVP.