von Willebrand disease (vWD)

What is von Willebrand disease?

von Willebrand Disease (vWD) is the most common type of inherited bleeding disorder. People with vWD have a problem with a protein in their blood called von Willebrand factor (VWF) that helps control bleeding. When a blood vessel is injured and bleeding occurs, VWF helps cells in the blood, called platelets, mesh together and form a clot to stop the bleeding. People with vWD do not have enough VWF, or it does not work the way it should. It takes longer for blood to clot and for bleeding to stop.

vWD is generally less severe than other bleeding disorders. Many people with vWD may not know that they have the disorder because their bleeding symptoms are very mild. For most people with vWD, the disorder causes little or no disruption to their lives except when there is a serious injury or need for surgery. However, with all forms of vWD, there can be bleeding problems. It is estimated that about 1 in 1000 of the world’s population suffers from vWD, but because many people have only very mild symptoms, only a small number of them know they have it. with the majority of people with vWD have not been diagnosed.

How vWD is passed from gereration to generation

Types of vWD

There are three main types of vWD. Within each type, the disorder can be mild, moderate, or severe. Bleeding symptoms can be quite variable within each type depending in part on the VWF activity. It is important to know which type of vWD a person has, because treatment is different for each type.

Type 1 vWD is the most common form. People with Type 1 vWD have lower than normal levels of VWF. Symptoms are usually very mild. Still, it is possible for someone with Type 1 vWD to have serious bleeding.
*Most common and mildest type (60-80% of patients)^1
*von Willebrand factor (VWF) works normally but there is not enough of it
*Symptoms are usually mild, but it is possible to have serious bleeding

Type 2 vWD involves a defect in the VWF structure. The VWF protein does not work properly, causing lower than normal VWF activity. There are different Type 2 vWD defects. Symptoms are usually moderate.
*Affects approximately 10-30% of patients¹
*There are different subtypes of Type 2 VWD depending on the type of VWF defect (2A, 2B, 2M, and 2N)
*Symptoms are usually moderate, but serious bleeding is possible

Type 3 vWD is usually the most serious form. People with Type 3 vWD have very little or no VWF. Symptoms are more severe. People with Type 3 vWD can have bleeding into muscles and joints, sometimes without injury.
*Rarest type. Affects approximately 1-5% of patients¹*Very little or no VWF is present. Low levels of FVIII
*Most serious. Symptoms are usually more severe. It is possible for people with Type 3 to have more serious bleeding issues, such as bleeding into muscles or joints, sometimes without an injury

Symptoms of von Willebrand Disease (VWD)

The most common symptom of VWD is bleeding (see description below). Many people have such mild symptoms they do not even know they have the disorder.²

Bleeding symptoms of VWD²

• Frequent, large bruises from minor bumps or injuries
• Frequent or hard-to-stop nosebleeds
• Extended bleeding from the gums after an invasive dental procedure (i.e. extractions)
• 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 surgery or childbirth

Heavy menstrual bleeding is often the main symptom of VWD in women. Doctors call this menorrhagia (men-o-RA-je-a). However, just because a woman has heavy menstrual bleeding doesn’t mean she has VWD.² It is important to review a detailed bleeding history with your doctor.  

Symptoms of menorrhagia that might suggest VWD²

• 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

1. Mannucci P. Treatment of von Willebrand’s disease. N Engl J Med. 2004;351:683-694.
2. National Heart Lung and Blood Institute. von Willebrand Disease. Last Update 2010. Available at: http://www.nhlbi.nih.gov/health/dci/Diseases/vWD/vWD_All.html. Accessed April 12, 2010.