Acquired von Willebrand disease (avWD) arises because of mechanisms that destroy, decrease, absorb, or clear von Willebrand factor (vWF). A 59-year-old man presented with a 3-year history of recurrent gastrointestinal bleeding. Laboratory workup revealed a prolonged platelet function assay-100. The vWF antigen was decreased, and a low vWF immunofunctional activity/antigen ratio, low collagen binding/antigen ratio, and decreased intermediate and high molecular weight multimers were noted. The patient had no high-shear stress conditions, and an antibody-mediated process was suspected. A vWF mixing study showed complete correction of vWF activity, suggesting no direct functional inhibitor. The patient was given a bolus of vWF concentrate with serial measurements of vWF; the vWF half-life was 2.5 hours. The vWF propeptide/antigen ratio was 4:1, supporting a diagnosis of aVWD resulting from increased antibody-mediated vWF clearance. This case study emphasizes the laboratory's role in the diagnosis and treatment of rare, overlooked acquired bleeding disorders.
Keywords: acquired von Willebrand disease; bleeding disorders; clotting; coagulation; coagulation testing; von Willebrand factor activity testing.
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