Learn About Von Willebrand Disease

What is the definition of Von Willebrand Disease (VWD)?

Von Willebrand disease is the most common hereditary bleeding disorder.

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What are the alternative names for Von Willebrand Disease (VWD)?

Bleeding disorder - von Willebrand

What are the causes of Von Willebrand Disease (VWD)?

Von Willebrand disease is caused by a deficiency or poor functioning of von Willebrand factor. Von Willebrand factor helps blood platelets clump together and stick to the blood vessel wall, which is necessary for normal blood clotting. There are several types of von Willebrand disease.

A family history of a bleeding disorder is the primary risk factor.

What are the symptoms of Von Willebrand Disease (VWD)?

Symptoms may include:

  • Abnormal menstrual bleeding
  • Bleeding of the gums
  • Bruising
  • Nosebleeds
  • Skin rash

Note: Most women with heavy or prolonged menstrual bleeding do not have von Willebrand disease.

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What are the current treatments for Von Willebrand Disease (VWD)?

Treatment may include DDAVP (desamino-8-arginine vasopressin). It is a medicine to raise von Willebrand factor level and reduce the chances for bleeding.

However, DDAVP does not work for all types of von Willebrand disease. Tests should be done to determine what type of von Willebrand you have. If you are going to have surgery, your doctor may give you DDAVP before surgery to see if your von Willebrand factor levels increase.

The drug Alphanate (antihemophilic factor) is approved to decrease bleeding in people with the disease who must have surgery or any other invasive procedure.

Blood plasma or certain factor VIII preparations may also be used to decrease bleeding.

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What is the outlook (prognosis) for Von Willebrand Disease (VWD)?

Bleeding may decrease during pregnancy. Women who have this condition usually do not have excessive bleeding during childbirth.

This disease is passed down through families. Genetic counseling may help prospective parents understand the risk for their children.

What are the possible complications of Von Willebrand Disease (VWD)?

Bleeding may occur after surgery or when you have a tooth pulled.

Aspirin and other nonsteroidal anti-inflammatory drugs (NSAIDs) can make this condition worse. Do not take these medicines without first talking to your health care provider.

When should I contact a medical professional for Von Willebrand Disease (VWD)?

Contact your provider if bleeding occurs without reason.

If you have von Willebrand disease and are scheduled for surgery or are in an accident, be sure you or your family tell the providers about your condition.

Blood clot formation
Blood clots
What are the latest Von Willebrand Disease Clinical Trials?
ATHN Transcends: A Natural History Cohort Study of the Safety, Effectiveness, and Practice of Treatment in People With Non-Neoplastic Hematologic Disorders

Summary: In parallel with the growth of American Thrombosis and Hemostasis Network's (ATHN) clinical studies, the number of new therapies for all congenital and acquired hematologic conditions, not just those for bleeding and clotting disorders, is increasing significantly. Some of the recently FDA-approved therapies for congenital and acquired hematologic conditions have yet to demonstrate long-term safet...

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A Phase 3, Prospective, Open-label, Uncontrolled, Multicenter Study on Efficacy and Safety of Prophylaxis With rVWF in Children Diagnosed With Severe Von Willebrand Disease

Summary: The main aim of the study is to evaluate the effectiveness of prophylaxis with recombinant von Willebrand factor (rVWF) in children. This study will enroll those participants who have been previously treated with VWF product or with a plasma-derived VWF (pdVWF) product. In this study, participants will be treated with rVWF for 12 months. During the study, participants will visit the study clinic 6...

What are the Latest Advances for Von Willebrand Disease?
Systemic bevacizumab as salvage therapy for persistent severe bleeding and anemia in heyde syndrome following aortic valve replacement.
Anagrelide for platelet-directed cytoreduction in polycythemia vera: Insights into utility and safety outcomes from a large multi-center database.
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Efficacy and Safety of Ombitasvir/Paritaprevir/ Ritonavir + Dasabuvir ± Ribavirin Combinations in Patients with Genotype 1 Hepatitis C and Inherited Bleeding Disorders.
Who are the sources who wrote this article ?

Published Date: January 25, 2022
Published By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

What are the references for this article ?

Flood VH, Scott JP. Von Willebrand disease. In: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Textbook of Pediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020:chap 504.

James P, Rydz N. Structure, biology, and genetics of von Willebrand factor. In: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Basic Principles and Practice. 7th ed. Philadelphia, PA: Elsevier; 2018:chap 138.

Neff AT. Von Willebrand disease and hemorrhagic abnormalities of platelet and vascular function. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 26th ed. Philadelphia, PA: Elsevier; 2020:chap 164.

Samuels P. Hematologic complications of pregnancy. In: Landon MB, Galan HL, Jauniaux ERM et al, eds. Gabbe's Obstetrics: Normal and Problem Pregnancies. 8th ed. Philadelphia, PA: Elsevier; 2021:chap 49.