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Von Willebrand disease

Von Willebrand disease is a condition that can cause extended or excessive bleeding. The condition is most often inherited but in rare cases may develop later in life.

The cause of von Willebrand disease is a deficiency in or impairment of a protein called von Willebrand factor, an important component in your blood-clotting process. In general, it takes longer for people with von Willebrand disease to form clots and stop bleeding when they're cut.

Treatment of von Willebrand disease focuses on stopping or preventing bleeding episodes, typically by using medications. With the right treatment, most people with von Willebrand disease can lead relatively normal, healthy lives.

Symptoms Causes Risk factors Complications Prevention

In many people with von Willebrand disease (vWD), the signs are mild or they may be absent altogether. When signs occur, their intensity can vary from one person to another. Von Willebrand disease is often challenging to diagnose, especially in milder cases.

Abnormal bleeding is the most common sign of von Willebrand disease, although it may be present at only moderate levels.

The abnormal bleeding associated with von Willebrand disease may occur as:

  • Recurrent and prolonged nosebleeds
  • Bleeding from the gums
  • Increased menstrual flow
  • Excessive bleeding from a cut or following a tooth extraction or other dental procedure
  • Blood in the stool or urine
  • Bleeding from shaving with a razor or other similarly minor injury

People with von Willebrand disease may also experience:

  • Easy bruising
  • Bruises with lumps that form underneath the skin

Some people may realize that they have a bleeding disorder only after a surgical procedure or serious trauma in which excessive bleeding occurs.

Signs and symptoms of von Willebrand disease in women

Heavy menstrual bleeding is often the main sign of von Willebrand disease in women. However, women with von Willebrand disease may go undiagnosed because they're not overly concerned about their prolonged or heavy menstrual bleeding. And doctors may overlook heavy menstrual bleeding as a possible indicator of von Willebrand disease.

Signs and symptoms of an abnormally heavy period that may indicate von Willebrand disease include:

  • The presence in your menstrual flow of blood clots greater than 1 inch (2.5 centimeters) in diameter
  • The need to change your menstrual pad or tampon more often than hourly
  • The need to use double sanitary protection to control menstrual flow
  • Symptoms of anemia, including tiredness, fatigue or shortness of breath

When to see a doctor

Contact your doctor if you're experiencing extended or severe bleeding from any source.

If you're scheduled to have surgery (including dental procedures), make sure your doctor or dentist knows that you have von Willebrand disease, which can increase the likelihood of postsurgical bleeding. Also be sure to mention if anyone in your family has a history of excessive bleeding.

Consider wearing a medical ID bracelet noting that you have von Willebrand disease in case you are in an accident and are taken to an emergency room. Also carry a medical alert card in your wallet.

The usual cause of von Willebrand disease is an inherited defect in the gene that controls von Willebrand factor, a protein that plays a key role in your blood-clotting process. When von Willebrand factor is scarce — or not functioning properly because of structural abnormalities — small blood cells called platelets cannot stick together properly, nor can they attach themselves normally to the blood vessel walls when an injury has occurred. The result is interference with the clotting process, and uncontrolled bleeding may persist.

Von Willebrand factor carries an additional substance, called factor VIII, that helps stimulate clotting. Many people with von Willebrand disease also have low levels of factor VIII.

Rarely, von Willebrand disease can develop later in life in people who didn't inherit an abnormal gene from a parent. This is known as acquired von Willebrand disease. The exact cause of this type of von Willebrand disease isn't clear. It may be an autoimmune disease, or it may be linked to a slow thyroid gland (hypothyroidism) or to certain medications, such as the anti-seizure medication valproic acid (Depakene) or the antibiotic ciprofloxacin (Cipro).

A family history of von Willebrand disease is the leading risk factor. A parent can pass the abnormal gene for the disease to his or her child.

Most cases are "autosomal dominant inherited" disorders, which means you only need an abnormal gene from one parent to be affected. If you have the gene for von Willebrand disease, you have a 50 percent chance of transmitting this gene to your offspring.

The most severe form of the condition (type 3) is "autosomal recessive," which means both of your parents have to pass an abnormal gene to you.

Complications of von Willebrand disease may include:

  • Anemia. Women who experience heavy menstrual bleeding can develop iron deficiency anemia.
  • Swelling and pain. If abnormal bleeding occurs in the joints or soft tissue, swelling and severe pain can result.
  • Death from bleeding. When abnormal bleeding can't be controlled, it can become life-threatening and needs emergency medical attention.

Because von Willebrand disease is usually an inherited disorder, consider having genetic counseling if you have a family history of this condition and you're planning to have children. If you carry the defective gene for von Willebrand disease, you can pass it on to your offspring, even if you don't have symptoms.

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