Background

- most common inherited bleeding disorder

- vWF is cofactor for platelet adhesion and carrier protein for factor VIII

- Three types: I (mild), II (4 subtypes exist), III (severe)

Clinical / Evaluation

- Patients often present with skin and mucosal bleeding - epistaxis, dental bleeding, menorrhagia, easy bruising

- Hemarthrosis uncommon

- PT and PTT usually normal

- Platelet count normal

- Bleeding time prolonged

- vWF activity level low

Treatment Options

- DDAVP = stimulates endothelial cells to secrete stored vWF, available in IV or SC or IN forms

Type 1 and most of Type 2s will respond to DDAVP. Patients with Type 3 disease UNLIKELY to respond to DDAVP

- Factor VIII and vWF concentrate (Humate-p)

Patients with severe Type 1, Type 3 and many with 2A, 2B and 2M disease will reqire replacement therapy

- Platelet transfusion if no response to plasma products

REFERENCES:

Tintinalli JE, Stapczynski JS, Ma OJ, Yealy DM, Meckler GD, Cline DM. Tintinalli's Emergency Medicine: A Comprehensive Study Guide. 8th ed. New York, NY: McGraw-Hill Education LLC, 2016.