Von Willebrand disease

Overview 
Theory 
Diagnosis 
Management 
Follow up 
Resources 

Case history

A 24-year-old woman presents to the emergency department 8 weeks postnatal with heavy vaginal bleeding, fatigue, and light-headedness. This was her first pregnancy. She has a history of heavy menstrual bleeding since menarche and iron-deficiency anaemia. She had no bleeding symptoms during her pregnancy, and her vaginal bleeding was not excessive in the first few days after delivery, but it has continued since the delivery and in the past week has increased in flow. Her past medical history is remarkable for an appendectomy at age 14 years without bleeding complications, but she had to return to the oral surgeon for suturing after wisdom tooth extraction at age 16 years. Her family history is remarkable for a sister with heavy menses. Her father had recurrent nosebleeds as a child and had several cauterisations as therapy.

Other presentations

Patients with von Willebrand disease (VWD) often have excessive mucocutaneous bleeding (including heavy menstrual bleeding, epistaxis, and easy bruising).[7][8] Patients may also have excessive bleeding from minor trauma/wounds or surgery (e.g., tonsillectomy, dental extraction).[7][8][9] Patients may have haematuria or central nervous system bleeding, but these are less common.[7][8][9][10][11]

Bleeding symptoms are usually more severe in type 2 and type 3 VWD than type 1 VWD, and may begin at an earlier age.[7][8][9] Severity of bleeding symptoms correlates with the reduction of von Willebrand factor levels and VWF activity. In type 3 VWD, FVIII is also severely decreased and may be low enough to put the patient at risk for severe bleeding symptoms more commonly seen in haemophilia A (e.g., joint bleeding [haemarthrosis], gastrointestinal bleeding).[8][9][12] Recurrent gastrointestinal bleeding may be a significant medical problem, particularly in older patients.

Patients with type 2B VWD often have mild to moderate thrombocytopenia.[13] A diagnosis of type 2B VWD may follow an incidental finding of thrombocytopenia, particularly in pregnancy, which tends to make the platelet count fall further.[14]

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