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Von Willebrand Disease

Von Willebrand disease (VWD) is a bleeding disorder that prevents your blood's ability to clot. It can cause heavy bleeding that’s hard to stop if you’re injured. With VWD, you either have low levels of the von Willebrand factor protein, or the protein doesn’t work well.

Von Willebrand factor protein helps platelets stick together and form blood clots, which stop bleeding.

Different gene mutations cause the different VWD types and subtypes, and each is treated differently. Types of von Willebrand Disease include:

• Type 1: Mildest and most common form of VWD. People with type 1 VWD have low von Willebrand factor levels and also could have low factor VIII levels. 75% of people with VWD have type 1.
• Type 2: The von Willebrand factor doesn't work well in those with type 2 VWD. Type 2 subtypes include:
  
  • 2A
  • 2B
  • 2M
  • 2N
• Type 3: People with type 3 VWD don’t usually have the von Willebrand factor protein, and they have low factor VIII levels. Type 3 is rare, but it is the most serious form of VWD.

Diagnosis

There is no one test that can diagnose VWD, but your doctor may recommend:

• Von Willebrand factor antigen: Measures the amount of von Willebrand factor in the blood
• Von Willebrand factor ristocetin cofactor activity: Shows how well your von Willebrand factor works
• Factor VIII clotting activity: Tests the clotting activity of factor VIII (Some people with 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 appear abnormal. It shows the structure of your von Willebrand factor and which type of VWD you have.
• Platelet function test: Measures how well your platelets are working

Symptoms

VWD may cause some of these symptoms, but other conditions may cause the same symptoms. See a doctor if you have any of these problems:

People with type 1 or type 2 VWD will have mild-to-moderate bleeding symptoms that include:

• Large bruises from minor bumps or injuries
• Nosebleeds
• Prolonged bleeding from the gums after dental work
• Heavy or prolonged periods in women
• Blood in stools and/or urine
• Heavy bleeding after a cut or other accident
• Heavy bleeding after surgery

People with type 3 VWD may have all of the symptoms listed above as well as severe bleeding episodes for no reason, which can be fatal if not treated immediately. Other symptoms for people with type 3 VWD include:

• Severe pain and swelling from bleeding in soft tissues or joints
• Heavy bleeding during a period is often is the main symptom of VWD in women, which is called menorrhagia. Menorrhagia is defined as:
• Blood clots larger than an inch in diameter
• Anemia (low red blood cell count) or low blood iron
• Changing pads or tampons more than once every hour
• Note that just because a woman has a heavy period doesn't mean she has VWD.

Treatment

Your type and severity of VWD determines your treatment. Some treatments options include:

• Taking medicines to:
  
  • Increase the amount of von Willebrand factor
  • Factor VIII released into your bloodstream
  • Replace von Willebrand factor
  • Prevent the breakdown of blood clots
  • Control heavy menstrual bleeding in women
• Taking the hormone desmopressin by injection or nasal spray. This releases von Willebrand factor and factor VIII into your bloodstream.
• Von Willebrand factor replacement therapy: Concentrated von Willebrand factor and factor VIII is injected through a vein in your arm
• Antifibrinolytic medicines: These prevent the breakdown of blood clots, such as after surgery or dental work

Treatments for women who have VWD with heavy periods include:

• Birth control pills
• Levonorgestrel intrauterine device: A birth control device containing the hormone progestin is placed in the uterus
• Aminocaproic acid or tranexamic acid: Reduces bleeding by slowing the breakdown of blood clots
• Desmopressin

These options are for women done having children or who do not wish to have children:

• Hysterectomy: Surgical removal of the uterus.
• Endometrial ablation: This destroys the lining of the uterus.

Risk Factors

• Family history of VWD

Related Links

Vanderbilt Hematology