A 45 year old female with no personal or family bleeding history was admitted to the hospital for gastrointestinal bleeding secondary to an ulcer. During the preoperative screening, an 8 cm anterior mediastinal mass was discovered on a routine chest x-ray (See Figure 1). PET/CT revealed hypermetabolism in the mass. Following surgery for the gastric ulcer, a small abdominal wall hematoma developed. An open biopsy of the mediastinal mass was performed, but was complicated by the formation of a left hemothorax (See Figure 2). Coagulation studies demonstrated a normal aPTT, PT, and platelet count. A platelet function assay (PFA)-100 was abnormal for both collagen/epinephrine and collagen/ADP suggesting a platelet function defect or von Willebrand disease (vWD). Platelet aggregation studies demonstrated decreased aggregation to ristocetin, but normal aggregation to the other agonists (arachidonic acid, collagen, epinephrine, and ADP). Additional coagulation studies performed revealed decreased FVIII activity, normal vWF:Ag level, but markedly reduced vWF:RCo activity (See Table 1- Lab). Clinically, bleeding continued despite DDAVP treatment, but was finally controlled with the administration of intravenous FVIIa. Maintenance therapy with FVIII/vWF:RCo (Humate-P) concentrates was attempted but bleeding continued. Further testing revealed elevated FVIII, vWF:Ag and vWF:RCo levels (See Table 2- Lab). Finally, complete resection of the mediastinal mass was performed which resulted in complete correction of the bleeding and coagulation disorder.
 Figure 1
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 Figure 2
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