Bleeding Disorders Overview | Hemophilia | Inhibitors | von Willebrand Disease

Von Willebrand disease (pron. vil'ĕ-brahnt) is an inherited disorder characterized by bleeding from mucous membranes (mouth, nose, throat, GI tract) and skin surfaces. The most common symptoms of von Willebrand disease (vWD) are excessive bruising, nosebleeds, heavy menstrual cycles (lasting more than 7 days), and excessive bleeding following surgery, childbirth, or tooth extraction.

The condition is caused by a defect or deficiency of a clotting protein called von Willebrand factor. This glue-like protein helps platelets in the blood stick together to form a plug over the site of an injury. Since people with vWD do not form a complete plug, bleeding continues longer.

Von Willebrand disease is different from hemophilia in that it affects males and females equally. It’s the most common inherited blood condition, and approximately 1 to 3 percent of the population is affected.

It doesn’t show up in routine blood tests. To diagnose vWD, a physician needs to order special tests. A person suspected of having vWD or another blood condition can be referred to a specialist hematologist or to a hemophilia treatment center.

A medical evaluation to diagnose vWD includes the individual’s medical history, the family medical history, a physical examination, and specific blood tests. Levels of von Willebrand factor can vary, so lab tests may have to be repeated.

 Classification

 vWD or Factor Level

 What to Expect

Type 1
  • Most common
  • Reduced levels of von Willebrand factor

Bleeding symptoms are usually mild.

Type 2

  • The von Willebrand factor doesn’t work properly

Patients usually have mild to moderate symptoms.

Type 3

  • Rare
  • Absence or very low levels of von Willebrand factor and factor VIII

Patients can experience deep and extensive tissue bleeding, similar to hemophilia. Similar precautions apply.

The choice of treatment will be directed by a hematologist or physician and will depend on the type of vWD present. Treatments include:

  • Amicar®. This medication is available in tablet and liquid form. It’s often used to treat bleeding in the mouth or nose, as well as heavy menstrual bleeding.
  • Desmopressin acetate. This medication is available as a concentrated nasal spray (Stimate®) or in injectable form (DDAVP). It stimulates the release of von Willebrand factor from storage sites in the lining of the blood vessels.
  • Clotting factor concentrates containing von Willebrand factor and factor VIII. These treatments must be given intravenously.