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What Is von Willebrand Disease?

Von Willebrand disease (VWD) is a bleeding disorder. It affects your blood's ability to clot. If your blood doesn't clot, you can have heavy, hard-to-stop bleeding after an injury. The bleeding can damage your internal organs. Rarely, the bleeding may even cause death.

In VWD, you either have low levels of a certain protein in your blood or the protein doesn't work well. The protein is called von Willebrand factor, and it helps your blood clot.

Normally, when one of your blood vessels is injured, you start to bleed. Small blood cell fragments called platelets (PLATE-lets) clump together to plug the hole in the blood vessel and stop the bleeding. Von Willebrand factor acts like glue to help the platelets stick together and form a blood clot.

Von Willebrand factor also carries clotting factor VIII (8), another important protein that helps your blood clot. Factor VIII is the protein that's missing or doesn't work well in people who have hemophilia, another bleeding disorder.

VWD is more common and usually milder than hemophilia. In fact, VWD is the most common inherited bleeding disorder. It occurs in about 1 out of every 100 to 1,000 people. VWD affects both males and females, while hemophilia mainly affects males.

Types of von Willebrand Disease

The three major types of VWD are called type 1, type 2, and type 3.

Type 1

People who have type 1 VWD have low levels of von Willebrand factor and may have low levels of factor VIII. Type 1 is the mildest and most common form of VWD. About 3 out of 4 people who have VWD have type 1.

Type 2

In type 2 VWD, the von Willebrand factor doesn't work well. Type 2 is divided into subtypes: 2A, 2B, 2M, and 2N. Different gene mutations (changes) cause each type, and each is treated differently. Thus, it's important to know the exact type of VWD that you have.

Type 3

People who have type 3 VWD usually have no von Willebrand factor and low levels of factor VIII. Type 3 is the most serious form of VWD, but it's very rare.

Overview

Most people who have VWD have type 1, a mild form. This type usually doesn't cause life-threatening bleeding. You may need treatment only if you have surgery, tooth extraction, or trauma. Treatment includes medicines and medical therapies.

Some people who have severe forms of VWD need emergency treatment to stop bleeding before it becomes life threatening.

Early diagnosis is important. With the proper treatment plan, even people who have type 3 VWD can live normal, active lives.

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June 01, 2011 Last Updated Icon

The NHLBI updates Health Topics articles on a biennial cycle based on a thorough review of research findings and new literature. The articles also are updated as needed if important new research is published. The date on each Health Topics article reflects when the content was originally posted or last revised.