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Key Features

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  • Easy bruising and epistaxis from early childhood

  • Menorrhagia

  • Prolonged platelet function analyzer (PFA)-100 (or bleeding time), normal platelet count, absence of acquired platelet dysfunction

  • Reduced amount or abnormal activity of von Willebrand factor (vWF)

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Clinical Findings

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  • A history of increased bruising and excessive epistaxis is often present

  • Prolonged bleeding also occurs with trauma or at surgery

  • Can develop in association with hypothyroidism, Wilms tumor, cardiac disease, kidney disease, or systemic lupus erythematosus, and in individuals receiving valproic acid

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Diagnosis

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  • Prothrombin time is normal

  • Activated partial thromboplastin time is sometimes prolonged

  • Prolongation of the PFA-100 or bleeding time is usually present

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Treatment

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  • Desmopressin

    • May be administered intravenously or subcutaneously

    • Typically elicits a three- to fivefold rise in plasma vWF

    • A high-concentration desmopressin nasal spray (150 μg/spray), different from the preparation used for enuresis, may be used

    • May cause fluid shifts, hyponatremia, and seizures

  • If further therapy is indicated, vWF-replacement therapy (eg, plasma-derived concentrate) is recommended

  • Antifibrinolytic agents (eg, ε-aminocaproic acid) may be useful for control of mucosal bleeding

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