Skip banner links and go to contentU.S. Department of Health & Human Services * National Institutes of Health
National Heart, Lung, and Blood Institute:  Diseases and Conditions Index
Tell us what you think about this site
  Enter keywords to search this site. (Click here for Search Tips)  
U.S. Department of Health & Human Services National Institutes of Health Diseases and Conditions Index NIH Home NHLBI Home About This Site NHLBI Home NHLBI Home Link to Spanish DCI Tell us what you think
 DCI Home: Blood Diseases: von Willebrand Disease: What Is ...

      von Willebrand Disease
Skip navigation and go to content
What Is ...
Causes
Signs & Symptoms
Diagnosis
Treatments
Living With
Key Points
Links
 

What Is von Willebrand Disease?

Von Willebrand disease (VWD) is an inherited 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 or even be life threatening, although this is rare.

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

Normally, when one of your blood vessels is injured, you start to bleed. Small blood cells 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 inactive or missing in hemophilia, another clotting disorder.

VWD is more common and usually milder than hemophilia. In fact, VWD is the most common of all the inherited bleeding disorders. 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

There are three major types of VWD.

Type 1

In type 1 VWD, you have a low level of the von Willebrand factor, and you may have lower levels of factor VIII than normal. This is the mildest and most common form of the disease. 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 the way it's supposed to. Type 2 is divided into subtypes: 2A, 2B, 2M, and 2N. Different gene mutations cause each type, and each is treated differently. This makes knowing the exact type of VWD that you have very important.

Type 3

In type 3 VWD, you 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 with VWD have type 1, a mild form. This type usually doesn't cause life-threatening bleeding, and you may need treatment only if you have surgery, tooth extraction, or trauma. If you need treatment, medicines and medical therapies are used.

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

Early diagnosis is important. With the right treatment plan, even people with type 3 VWD can be helped to live normal, active lives.

August 2008


Next Causes


Email this Page Email all Sections Print all Sections Print all Sections of this Topic


Skip bottom navigation and go back to top
Department of Health and Human Services National Institutes of Health National Heart, Lung, and Blood Institute
Blood Diseases | Heart and Blood Vessel Diseases | Lung Diseases | Sleep Disorders
NHLBI Privacy Statement | NHLBI Accessibility Policy
NIH Home | NHLBI Home | DCI Home | About DCI | Search
About NHLBI | Contact NHLBI

Note to users of screen readers and other assistive technologies: please report your problems here.