Von Willebrand disease medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Medical Therapy
| Type | Treatment | Additional/Alternative treatement |
|---|---|---|
| Low vWF | Desmopressin administered intravenously 0.3μg per kilogram body weight,
intranasally (300μg|150μg per nostril; in patients with body weight <50Kg, only one dose of 150μg or subcutaneously 0.3μg/kilogram |
Tranexamic acid 1g 3 to 4 times daily |
| 1 | Demospressin at same dose as above | Tranexamic acid 1g 3 to 4 times daily |
| 2 | Demospressin at same dose as above or vWF-Factor VIII or vWF concentrate | Tranexamic acid 1g 3 to 4 times daily |
| 3 | vWF-Factor VIII or vWF concentrate | Tranexamic acid 1g 3 to 4 times daily |
Desmopressin is contraindicated in patients with type 2B disease.
For women with heavy menstrual bleeding, the combined oral contraceptive pill may be effective in reducing bleeding or in reducing the length or frequency of periods. Prophylactic treatment is sometimes given for patients with vWD who are scheduled for surgery. They can be treated with human derived medium purity factor VIII concentrates complexed to vWF (antihemophilic factor, more commonly known as Humate-P) Mild cases of vWD can be trialled on desmopressin (1-desamino-8-D-arginine vasopressin, DDAVP) (desmopressin acetate, Stimate), which works by raising the patient's own plasma levels of vWF by inducing release of vWF stored in the Weibel-Palade bodies in the endothelial cells.