Von Willebrand Disease: Types, Symptoms, Diagnosis & Treatment

Von Willebrand disease (VWD) is the most common inherited bleeding disorder, caused by deficiency or dysfunction of von Willebrand factor (VWF), a protein essential for platelet adhesion and factor VIII stabilization.

Common Symptoms

• Easy bruising
• Prolonged bleeding from cuts, dental work, surgery
• Heavy menstrual bleeding (menorrhagia)
• Frequent nosebleeds (epistaxis)
• Bleeding gums
• Postpartum hemorrhage

Three Main Types

1. Type 1 (60–80%) – Partial quantitative VWF deficiency; usually mild.
2. Type 2 (15–30%) – Qualitative defects (subtypes 2A, 2B, 2M, 2N); variable severity.
3. Type 3 (<5%) – Near-complete VWF absence; severe bleeding, similar to hemophilia.

Diagnosis

VWF antigen, VWF activity (ristocetin cofactor), factor VIII level, VWF multimer analysis. Repeat testing may be needed (VWF rises with stress, inflammation, pregnancy, OCPs).

Treatment

• Desmopressin (DDAVP) – Releases endothelial VWF stores; effective for most Type 1, some Type 2. Not for Type 2B.
• Tranexamic acid / aminocaproic acid – Antifibrinolytics; adjunct for mucosal bleeding.
• VWF/FVIII concentrates – For Type 3, severe Type 2, or DDAVP non-responders; perioperative prophylaxis.
• Hormonal therapy – For menorrhagia (combined OCPs, levonorgestrel IUD).

With proper management, patients lead active lives. Care by a hematologist experienced in bleeding disorders is ideal.