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Acquired von Willebrand disease (aVWD) is rare disease and is associated with a variety of underlying diseases. We report a case of aVWD in the setting of multiple myeloma. The patient was a 63-year-old female with breast cancer in remission who was admitted for symptomatic anemia. She was diagnosed with multiple myeloma. She also had subcutaneous bleeding before admission. Laboratory studies revealed isolated prolongation of the activated partial thromboplastin time, which corrected in a mixing study. Her factor VIII activity, von Willebrand factor (VWF) Ag, and VWF activity were low. VWF multimer study confirmed the patient had aVWD. The treatment of aVWD is discussed in this article, including the treatment of underlying diseases, and acute management in emergent situations. An intriguing question in this case is whether the patient's multiple myeloma is a chemotherapy-induced hematological malignancy or a second primary malignancy.

von Willebrand disease (VWD) is associated with development of gastrointestinal (GI) vascular malformations that lead to chronic GI bleeding. Conventional management, including von Willebrand factor concentrate replacement and endoscopic ablation or bowel resection, does not consistently reduce hemorrhage. We describe three patients with VWD for whom conventional management failed to control GI bleeding. We retrospectively reviewed medical records of patients with VWD and GI bleeding. After patients began treatment with danazol, we observed long-term reductions in GI bleeding and packed red blood cell transfusion requirements. One patient had severe liver toxicity and was found to have concomitant primary biliary cirrhosis. Danazol use may be considered in patients with VWD and GI bleeding due to angiodysplasia that otherwise fails to respond to conventional treatment; the primary aim of treatment is to reduce transfusion dependence. The benefits are variable and possibly transient. Monitoring for toxicity is important when this treatment is pursued.

Acquired von Willebrand syndrome (AVWS) is a rare bleeding disorder that typically presents as mucocutaneous bleeding in individuals with no personal or family history of bleeding disorder. Here we present a case in which a patient presented with profound epistaxis and was found to have AVWS in the setting of monoclonal gammopathy of undetermined significance (MGUS).

von Willebrand disease (VWD) is considered to be the most common inherited bleeding disorder. VWD is diagnosed following a clinical and physical review, with personal and familial evidence of (primarily mucocutaneous) bleeding, and confirmed by laboratory testing. The latter typically entails initial plasma testing of factor VIII coagulant, von Willebrand factor (VWF) protein ('antigen') and VWF function which has classically been assessed using the ristocetin cofactor (VWF:RCo) assay. More recent attention has focussed on other functional VWF assays, such as collagen binding and so-called 'VWF activity' assays, as possible replacements to the VWF:RCo, or as supplementary tests of VWF 'function'. Additional laboratory testing can comprise a battery of confirmatory and VWD-type assisting assays, including VWF:multimer and von Willebrand factor VIII binding. This review aims to update knowledge of current VWD diagnostics with a particular emphasis on 'functional' VWF assays.

Heyde's syndrome is characterized by iron deficiency anemia due to gastrointestinal bleeding and calcific aortic stenosis. Patients with this syndrome have a bleeding diathesis due to a loss of the largest multimers of von Willebrand factor (vWF). Here we present a case of Heyde's syndrome diagnosed with abnormal closure times and normal vWF Ristocetin cofactor activity (vWF:Rco). In this case, a 79-year-old man with known aortic stenosis and recurrent gastrointestinal bleeding was cured of a life-threatening hemorrhage after the replacement of his stenotic aortic valve. Factor VIII activity (1.53  IU/ml), vWF antigen (1.26  IU/ml), vWF:Rco (1.11  IU/ ml), and the ratio of vWF antigen/vWF:Rco (0.88) were all within normal limits. Instead, to prove a defect in platelet aggregation, closure times measured with collagen/ADP and collagen/epinephrine were abnormal (>300  s). Postoperatively, these closure times normalized. What is unique about our current report is that we measured both vWF:Rco and closure times, the two readily available assays in most coagulation laboratories. vWF:Rco is a standard assay for measuring platelet activity but may miss defects in platelet aggregation that are only seen under high shear stress. As the closure times can detect such defects, it is perhaps more representative than traditional assays, and in situations such as our case, closure times may be the only method by which subtle abnormalities in vWF function could be detected.