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Often severe mucosal and postoperative bleeding
Bleeding due to defective platelets is classically mucocutaneous, but is not limited to mucocutaneous surfaces
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Presence of abnormally large platelets (approaching the size of red cells)
Moderate thrombocytopenia
Prolonged bleeding time
Platelet aggregation studies show a marked defect in response to ristocetin, whereas aggregation in response to other agonists is normal
Addition of normal platelets corrects the abnormal aggregation
Platelet flow cytometry confirms the diagnosis
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Platelet transfusion when necessary
Desmopressin acetate (DDAVP), antifibrinolytic agents, and recombinant human activated factor VII also have been used successfully