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Von Willebrand disease

Overview

Von Willebrand disease is a lifelong bleeding disorder in which your blood doesn't clot well. Most people with the disease are born with it, though its warning signs may not show up for years. Some people may suspect they have a bleeding disorder when they have heavy bleeding after a dental procedure or, for women, during a menstrual period.

Most people with this condition inherited it from a parent. They have a faulty gene that causes problems with a protein important to the blood-clotting process.

Von Willebrand disease can't be cured. But with good treatment and self-care, most people with this disease can lead active lives.

Symptoms

Many people with von Willebrand disease don't know it because the signs are mild or absent. The most common sign of the condition is abnormal bleeding. The severity of the bleeding varies from one person to another.

If you have von Willebrand disease, you might experience:

  • Excessive bleeding from an injury or after surgery or dental work
  • Nosebleeds that don't stop within 10 minutes
  • Heavy or long menstrual bleeding
  • Blood in your urine or stool
  • Easy bruising or lumpy bruises

Signs and symptoms of von Willebrand disease in women

Signs and symptoms of a 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 long or hard-to-stop bleeding.

Causes

The usual cause of von Willebrand disease is an inherited abnormal gene that controls von Willebrand factor, a protein that plays a key role in blood-clotting. When you have low levels of this protein or it doesn't work as it should, 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, sometimes, uncontrolled bleeding.

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. This is also one of the substances involved in another inherited clotting disorder called hemophilia. But unlike hemophilia, which mainly affects males, von Willebrand disease affects males and females and is usually milder.

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, and it's likely caused by another medical condition.

Von Willebrand disease has several types:

  • Type 1. In this most common form of von Willebrand disease, levels of von Willebrand factor are low. In some people, levels of factor VIII also are low. Signs and symptoms are usually mild.
  • Type 2. In this type, which has several subtypes, the von Willebrand factor you do have doesn't function properly. Signs and symptoms tend to be more significant.
  • Type 3. In this rare type, von Willebrand factor is absent and levels of factor VIII are low. Signs and symptoms may be severe, such as bleeding into the joints and muscles.
  • Acquired von Willebrand disease. This type isn't inherited from your parents. It develops later in life.

Risk factors

The main risk factor for von Willebrand disease is having a family history of it. 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.

Autosomal dominant inheritance pattern

In an autosomal dominant disorder, the mutated gene is a dominant gene located on one of the nonsex chromosomes (autosomes). You need only one mutated gene to be affected by this type of disorder. A person with an autosomal dominant disorder — in this case, the father — has a 50 percent chance of having an affected child with one mutated gene (dominant gene) and a 50 percent chance of having an unaffected child with two normal genes (recessive genes).

Autosomal dominant inheritance pattern

In an autosomal dominant disorder, the mutated gene is a dominant gene located on one of the nonsex chromosomes (autosomes). You need only one mutated gene to be affected by this type of disorder. A person with an autosomal dominant disorder — in this case, the father — has a 50 percent chance of having an affected child with one mutated gene (dominant gene) and a 50 percent chance of having an unaffected child with two normal genes (recessive genes).

Autosomal recessive inheritance pattern

To have an autosomal recessive disorder, you inherit two mutated genes, one from each parent. These disorders are usually passed on by two carriers. Their health is rarely affected, but they have one mutated gene (recessive gene) and one normal gene (dominant gene) for the condition. Two carriers have a 25 percent chance of having an unaffected child with two normal genes (left), a 50 percent chance of having an unaffected child who also is a carrier (middle), and a 25 percent chance of having an affected child with two recessive genes (right).

Complications

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. Rarely, someone with von Willebrand disease may experience uncontrolled bleeding that can be life-threatening and needs emergency medical attention.

Diagnosis

Because many people with von Willebrand disease have mild signs and symptoms, the condition can be difficult to diagnose. If you have any indication of a bleeding disorder, your doctor may refer you to a blood disorders specialist (hematologist).

To evaluate you for von Willebrand disease, your doctor will likely ask you detailed questions about your medical history and check for bruises or other signs of recent bleeding.

Your doctor will also likely recommend the following blood tests:

  • Von Willebrand factor antigen. This test determines the level of von Willebrand factor in your blood by measuring a particular protein.
  • Ristocetin cofactor activity. This test measures how well the von Willebrand factor works in your clotting process. Ristocetin, which is an antibiotic, is used in this laboratory testing.
  • Factor VIII clotting activity. This test shows whether you have abnormally low levels and activity of factor VIII.
  • Von Willebrand factor multimers. This test evaluates the specific structure of von Willebrand factor in your blood, its protein complexes (multimers) and how its molecules break down. This information helps identify the type of von Willebrand disease you have.

The results of these tests can fluctuate in the same person over time due to factors such as stress, exercise, infection, pregnancy and medications. So you may need to repeat some tests.

If you have von Willebrand disease, your doctor may recommend that family members undergo the same or similar tests to determine if this condition runs in your family.

Treatment

Even though von Willebrand disease is a lifelong condition with no cure, treatment can help prevent or stop bleeding episodes. Your treatment depends on:

  • The type and severity of your condition
  • How you've responded to previous therapy
  • Your other medications and conditions

Your doctor may suggest one or more of the following treatments to increase your von Willebrand factor, strengthen blood clots or, in women, control heavy menstrual bleeding:

  • Desmopressin. This medication is available as an injection (DDAVP) or nasal spray (Stimate). It's a synthetic hormone, similar to the natural hormone vasopressin. It controls bleeding by stimulating your body to release more von Willebrand factor already stored in the lining of your blood vessels. DDAVP is usually effective in people with type 1 and some subtypes of type 2 disease.

    Many doctors consider DDAVP the first treatment to use in the management of von Willebrand disease. Some women use the nasal spray (Stimate) at the beginning of their menstrual periods to control excessive bleeding. It can also be effective when used before a minor surgical procedure.

  • Replacement therapies. These include infusions of prepared doses of concentrated blood-clotting factors containing von Willebrand factor and factor VIII (Humate-P, others). These therapies can be useful in all disease types. Your doctor may recommend them if DDAVP isn't an option for you or was ineffective.

    Another replacement therapy approved by the FDA for treating adults 18 and older is a genetically engineered (recombinant) von Willebrand factor product (Vonvendi). Because recombinant factor is made without plasma, it may reduce the risk of a viral infection or allergic reaction.

  • Contraceptives. For women, these can be useful for controlling heavy bleeding during menstrual periods. The estrogen hormones present in birth control pills can boost levels of von Willebrand factor and factor VIII activity. This effect is likely available with birth control patches, though further study is needed to confirm it.
  • Clot-stabilizing medications. These anti-fibrinolytic medications — such as aminocaproic acid (Amicar) and tranexamic acid (Cyklokapron, Lysteda, others) — can help stop bleeding by slowing the breakdown of blood clots. Doctors often prescribe these drugs before or after a surgical procedure or tooth extraction.
  • Drugs applied to cuts. A fibrin sealant (Tisseel VHSD) placed directly on a cut helps curtail bleeding. These substances are applied like a glue using syringes.

If your condition is mild, your doctor might recommend treatment only when you're undergoing surgery or dental work or when you've experienced trauma (in an automobile accident, for example).

Preparing for an appointment

You're likely to start by seeing your family doctor if you think you have a bleeding problem. But in some cases when you call to set up an appointment, you may be referred to a doctor who specializes in the diagnosis and treatment of bleeding disorders (hematologist).

If you're in the middle of a severe bleeding episode, your doctor may recommend immediate medical care.

Because appointments can be brief, and because there's often a lot of ground to cover, it's a good idea to be well-prepared. Here's some information to help you get ready, including what to expect from the doctor.

What you can do

  • Be aware of any pre-appointment restrictions. At the time you make the appointment, ask if you need to do anything in advance. You may need to follow dietary restrictions or fast for eight to 10 hours if blood tests are scheduled.
  • List any bleeding signs or symptoms you've had, including how often, its severity and for how long. For example, your doctor will want to know if you had a history of nosebleeds or easy bruising since childhood or — for women — if your periods have been extended and heavy since you first started menstruating.
  • List key personal information, including any major stresses or recent changes — both positive and negative — in your life. Include a short description of your typical daily diet. Also mention any contact sports you've played and whether you ever developed excessive bruising after playing.
  • Make a list of your key medical information, including other medical problems for which you're being treated. Be sure to mention if any close relatives have a history of easy bleeding. Also list any medications, vitamins or supplements you're taking, including aspirin, ibuprofen (Advil, Motrin IB, others) or naproxen (Aleve, Anaprox). If possible, bring all your medications with you in their original containers.
  • Take a family member or friend along. Sometimes it can be difficult to remember all of the information provided to you during an appointment. Someone who accompanies you may remember something that you missed or forgot.
  • List questions to ask your doctor.

For von Willebrand disease, some basic questions to ask your doctor include:

  • What's the most likely reason for my symptoms?
  • What treatment do you recommend, and how will you monitor whether it's working?
  • What are the possible side effects of the medications you're prescribing?
  • Does this condition increase my risk of any other medical problems?
  • What steps will I need to take to prevent problems from surgery or dental procedures?
  • Does this condition increase my risk of health problems during pregnancy and childbirth? Are treatment options available to reduce that risk?
  • Are my children or other close relatives at increased risk of this condition?
  • Do I need to avoid certain physical activities or types of exercise?
  • I have these other health conditions. How can I best manage these conditions together?

What to expect from your doctor

Your doctor may ask:

  • When did you first begin having episodes of prolonged or heavy bleeding?
  • How often do you have an episode of heavy or prolonged bleeding, and how long do these episodes typically last?
  • Do you bruise easily or have recurrent nosebleeds? Did these symptoms begin in childhood?
  • Have you ever had bleeding from a small wound that lasted more than 15 minutes or recurred during the week following the wound?
  • Have you ever had a nosebleed that lasted more than 10 minutes or needed medical attention?
  • For women, for how many days do your menstrual periods last? How often do you have to change your tampon or sanitary pad during a period? Do you ever notice blood clots in your menstrual flow?
  • Have you ever had blood in your stool that wasn't explained by a known medical problem, such as a stomach ulcer or colon polyp?
  • Have you ever needed medical attention for a bleeding problem during or after surgery, dental procedures, childbirth or injury?
  • Have you ever had anemia or needed a blood transfusion?
  • Have you been diagnosed or treated for other medical problems, including liver or kidney disease, a blood or bone marrow disorder, or an abnormal blood platelet count?
  • Do you take pain relievers, such as aspirin, ibuprofen or naproxen? What about blood-thinning medications, such as clopidogrel (Plavix), warfarin (Coumadin) or heparin?
  • Do you take an antidepressant, such as citalopram (Celexa), escitalopram (Lexapro), fluoxetine (Prozac, Sarafem), paroxetine (Paxil, Paxil CR, Pexeva, others) or sertraline (Zoloft)?
  • Does anyone in your family have a history of bleeding problems?

What you can do in the meantime

While you wait for your appointment, avoid pain relievers that may increase your risk of bleeding episodes, such as aspirin, ibuprofen and naproxen. If you need relief for routine aches and pains, try acetaminophen (Tylenol, others) instead.

Steer clear of contact sports associated with a high risk of bruising or injury, such as football and hockey. If you have any medical or dental procedures scheduled, tell your doctor or dentist about your history of heavy bleeding from minor injuries. If a scheduled procedure is not urgent, reschedule it until after you've been evaluated for a possible underlying bleeding disorder.

Lifestyle and home remedies

These self-care tips can help you manage your condition:

  • Switch pain relievers. To help prevent bleeding episodes, talk with your doctor before taking blood-thinning medications — such as aspirin, ibuprofen (Advil, Motrin IB, others) or naproxen (Aleve, Anaprox). Your doctor may instead recommend pain and fever relievers such as acetaminophen (Tylenol, others).
  • Tell your doctors and dentist. Let your doctors or dentist know that you have von Willebrand disease before you have any type of surgery, start a new medication or give birth. Also mention whether anyone in your family has a history of excessive bleeding.
  • Consider wearing a medical ID bracelet. Make sure it notes that you have von Willebrand disease, which will be helpful to medical personnel if you're ever in an accident or taken to an emergency room. Also carry a medical alert card in your wallet.
  • Be active and safe. Exercise as part of attaining or maintaining a healthy weight. Avoid activities that could cause bruising, such as football, wrestling and hockey.

Prevention

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.

Last updated: December 3rd, 2016

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