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von Willebrand disease (VWD)

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What is Von Willebrand disease (VWD)?

Von Willebrand disease (VWD) is a common bleeding disorder caused by inheriting an abnormal gene that controls a protein (called von Willebrand factor) that plays a key role in blood clotting.

People with Von Willebrand disease have low levels, defective, or zero von Willebrand factor in their blood.

What causes von Willebrand disease (VWD)?

The Von Willebrand gene (located on chromosome 12) codes for a specific blood-clotting protein called von Willebrand factor. This protein helps platelets stick together and adhere to the walls of blood vessels at the site of a wound. It is vital for the proper formation of blood clots, which is the way our body seals off damaged blood vessels and prevents further blood loss after injury. Von Willebrand factor also carries another blood-clotting protein, coagulation factor VIII.

People with Von Willebrand disease (VWD) have either abnormal or missing von Willebrand gene. There are four main types of VWD (three are hereditary and one is acquired):

  • Type 1 VWD: The most common type, found in 60% to 80% of people. Characterized by a quantitative deficiency of VWF; levels of VWF in the blood range from 20-50% or normal. Symptoms are usually mild.
  • Type 2 VWD: Found in 15%-30% of patients. Characterized by a qualitative deficiency in VWF. Four subtypes: type 2A, type 2B, type 2M and type 2N. Symptoms are mild to moderate.
  • Type 3 VWD: Found in 1%-10% of patients. Characterized by a severe quantitative defect or virtual absence of VWF; Symptoms are typically severe and include spontaneous bleeding episodes, often into joints and muscles.
  • Acquired VWD: Rare. May occur following diagnosis of an autoimmune disease (eg, lupus), heart disease, some types of cancer, or certain medications (eg, antibiotics, anticonvulsants, plasma expanders).

VWD affects up to 1% of the US population and occurs equally in men and women.

What are the symptoms of von Willebrand disease (VWD)?

Symptoms may vary depending on the type of von Willebrand disease (VWD) but may include:

  • Easy bruising
  • Excessive bleeding during and after invasive procedures (eg, tooth extractions, or surgery)
  • Frequent nosebleeds
  • Heavy menstrual bleeding
  • Hemorrhaging after childbirth.

How is von Willebrand disease (VWD) diagnosed?

See your doctor if you experience frequent or heavy bleeding. Your doctor will ask you about your history, conduct some blood tests, and refer you to a federally funded hemophilia treatment center or other specialist providers.

Note that levels of VWF fluctuate, and blood tests may need to be repeated before an accurate result is obtained because levels of VWF can rise due to stress, exercise, oral contraceptives, pregnancy, and hyperthyroidism.

How is von Willebrand disease (VWD) treated?

Treatment depends on the type and severity of VWD. Treatments may include:

  • Injectable or nasal desmopressin (DDAVP)
  • Recombinant VWF (Vonvendi)
  • Aminocaproic acid
  • Tranexamic acid.