Hematology and Oncology Book

http://www.fpnotebook.com/

Von Willebrand's Disease

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  1. Epidemiology
    1. Most common inherited Bleeding Disorder
    2. Mild Bleeding Disorder (often undiagnosed)
    3. Autosomal dominant disorder
  2. Pathophysiology
    1. Von Willebrand factor mediates platelet adhesion
    2. vWF Deficiency results in mucocutaneous bleeding
  3. Symptoms and Signs
    1. Severe Menorrhagia (common presentation in women)
    2. Postpartum Hemorrhage several days after delivery
  4. Labs
    1. Lab Results vary over time in each patient
    2. Partial Thromboplastin Time (PTT) prolonged
    3. Bleeding Time prolonged
    4. Platelet Function Closure Time (PFCT)
      1. Replaces Bleeding Time
  5. Management
    1. Synthetic hormone arginine vasopressin (DDAAVP)
      1. Indicated for surgery or trauma
    2. Cryoprecipitate

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