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Treatment 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 or even cause death, 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 cell fragments called platelets 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, another important protein that helps your blood clot. Factor VIII is the protein that's inactive or missing in people who have 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

There are three major types of VWD.

Type 1
In type 1 VWD, you have a low level of von Willebrand factor, and you may have lower than normal levels of factor VIII. This 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 the way it should. Type 2 is divided into subtypes: 2A, 2B, 2M, and 2N. Different gene mutations (changes) cause each type, and each is treated differently. So it's important to know the exact type of VWD that you have.

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.

Causes

Von Willebrand disease is almost always inherited. You can inherit type 1 or type 2 VWD if only one of your parents passes the gene on to you. You usually inherit type 3 VWD only if both of your parents pass the gene on to you. Your symptoms may be different from your parents' symptoms.

Some people have the genes for the disorder but don't have symptoms. However, they still can pass the genes on to their children.

Symptoms

The signs and symptoms of von Willebrand disease depend on the type and severity of the disorder. Many people have such mild symptoms that they don't know they have the disorder.

If you have type 1 or type 2 VWD, you may have the following mild-to-moderate bleeding symptoms:

  • Frequent, large bruises from minor bumps or injuries
  • Frequent or hard-to-stop nosebleeds
  • Extended bleeding from the gums after a dental procedure
  • Heavy or extended menstrual bleeding in women
  • Blood in your stools from bleeding in your intestines or stomach
  • Blood in your urine from bleeding in your kidneys or bladder
  • Heavy bleeding after a cut or other accident
  • Heavy bleeding after surgery

People who have type 3 VWD may have all of the symptoms listed above, as well as severe bleeding episodes for no reason. These bleeding episodes can be fatal if not treated right away. People who have type 3 VWD also may have bleeding into soft tissues or joints, causing severe pain and swelling.

Heavy menstrual bleeding is often the main symptom of VWD in women. Doctors call this menorrhagia. They define it as:

  • Bleeding with clots larger than about 1-inch in diameter
  • Anemia or low blood iron
  • The need to change pads or tampons more than every hour

However, just because a woman has heavy menstrual bleeding doesn't mean she has VWD.

Diagnosis

No single test can diagnose VWD. Your doctor may recommend a combination of blood tests to diagnose the disorder. These tests may include:

  • Von Willebrand factor antigen. This test measures the amount of von Willebrand factor in your blood.
  • Von Willebrand factor ristocetin cofactor activity. This test shows how well your von Willebrand factor works.
  • Factor VIII clotting activity. Some people who have VWD have low levels of factor VIII activity, while others have normal levels.
  • Von Willebrand factor multimers. This test is done if one or more of the first three tests are abnormal. It shows the structure of your von Willebrand factor. The test helps your doctor diagnose what type of VWD you have.
  • Platelet function test. This test measures how well your platelets are working.

 

Source

National Heart, Lung, and Blood Institute, USA.