von Willebrand Disease

What is von Willebrand Disease?

Von Willebrand disease is the most common hereditary bleeding disorder.

What causes von Willebrand Disease?

Von Willebrand disease is caused by a deficiency of von Willebrand factor. Von Willebrand factor helps blood platelets clump together and stick to the blood vessel wall, which is necessary for normal blood clotting. There are several types of von Willebrand disease.

A family history of a bleeding disorder is the primary risk factor.

What are the symptoms of von Willebrand Disease?

Symptoms may include:

  • Abnormal menstrual bleeding
  • Bleeding of the gums
  • Bruising
  • Nosebleeds
  • Skin rash

Note: Most women with heavy or prolonged menstrual bleeding do not have von Willebrand disease.

How is von Willebrand Diseased diagnosed?

Von Willebrand disease may be hard to diagnose. Low von Willebrand factor levels and bleeding do not always mean you have von Willebrand disease.

Tests that may be done to diagnose this disease include:

  • Bleeding time
  • Blood typing
  • Factor VIII level
  • Platelet function analysis
  • Platelet count
  • Ristocetin cofactor test
  • Von Willebrand factor specific tests

What is the treatment for von Willebrand Disease?

Treatment may include DDAVP (desamino-8-arginine vasopressin), a medicine to raise von Willebrand factor level and reduce the chances for bleeding.

However, DDAVP does not work for all types of von Willebrand disease. Tests should be done to determine what type of von Willebrand you have. If you are going to have surgery, your doctor may give you DDAVP before surgery to see if your von Willebrand factor levels increase.

The drug Alphanate (antihemophilic factor) is approved to decrease bleeding in people with the disease who must have surgery or any other invasive procedure.

Blood plasma or certain factor VIII preparations may also be used to decrease bleeding.