Pathophysiology and management of acquired von Willebrand syndrome

Acquired von Willebrand disease (AVWD) is a defect of coagulation occurring secondary to other pathologies, leading to abnormal von Willebrand factor (VWF) function. Multiple pathophysiological mechanisms are implicated, and broadly, these are categorized into immune (e.g., antibody-related clearance) and nonimmune causes (e.g., shear-related loss of high molecular weight multimers in complex cardiac anatomy or in patients on circulatory support devices). High index of suspicion is required for correct diagnosis. Evaluation consists of VWF activity and antigen levels, as well as multimer testing. Therapy should be geared toward correcting the underlying issue, as once the offending mechanism is removed, VWF levels go back to normal. VWF replacement and adjunctive therapies can be utilized to control bleeding in the interim.