Von Willebrand Disease


What is von Willebrand disease?

Von Willebrand disease is a common blood disorder that keeps your blood from clotting. This is an inherited disorder, meaning parents may pass the disorder on to their biological children. Healthcare providers treat this disorder with medication to help with blood clotting.

What happens if you have von Willebrand disease?

People with von Willebrand disease may bleed more than usual. For example, they may have frequent nosebleeds or bleed for a long time after getting a minor cut. Women or people designated female at birth (DFAB) may have heavy menstrual periods or heavy bleeding after giving birth. People who have the most serious form of von Willebrand disease may have bleeding into their joints or soft tissues that cause severe pain and swelling. Some people develop anemia.

How common is this condition?

Von Willebrand disease affects 1% of the U.S. population and is the most common bleeding disorder in the United States. Globally, von Willebrand disease affects an estimated 23 to 110 in 1 million people. The numbers vary because people may have bleeding issues, but aren’t diagnosed with von Willebrand disease. In some cases, people have had bleeding issues for many years before they have a firm diagnosis.

Is von Willebrand disease the same as hemophilia?

Von Willebrand is similar to hemophilia but typically causes less severe symptoms.

Symptoms and Causes

What are the symptoms of von Willebrand disease?

Many people with von Willebrand disease have the condition, but don’t have symptoms or have mild symptoms. People who have a more severe form of the condition may have the following symptoms:

  • Nosebleeds: These are nosebleeds that last longer than 10 minutes and happen five or more times a year.
  • Bleeding from a cut or other injury that lasts longer than 10 minutes.
  • Bruises: People with von Willebrand disease bruise easily. Their bruises are raised, meaning the bruises look like they’re swollen, and their bruises are larger than a quarter.
  • Iron-deficiency anemia: All anemia happens when you don’t have enough red blood cells. In iron-deficiency anemia, your body doesn’t have enough iron to make hemoglobin. Hemoglobin is the substance in your red blood cells that helps them carry oxygen.
  • Post-surgery bleeding: People with von Willebrand disease may have heavy bleeding after any surgery, including dental surgery.
  • Heavy periods (menstrual bleeding): This is bleeding that’s so heavy you need to change your pad or tampon every hour, or have bleeding that lasts longer than seven days.
  • Heavy bleeding after childbirth or miscarriage.
  • Blood in poop (stool): Blood in your poop or bleeding after pooping may be a symptom of other medical conditions. Talk to your healthcare provider if you have blood in your poop.
  • Blood in pee (hematuria): Talk to your healthcare provider if you notice blood when you pee, particularly if you have an urgent need to pee and there’s blood in your pee.

What causes von Willebrand disease?

Von Willebrand disease is a genetic disorder that happens when certain genes mutate, or change. In von Willebrand disease, genetic mutations affect your body’s ability to make normal von Willebrand factor. Factors are proteins that help your blood to clot.

You have von Willebrand factor in your plasma, platelets and walls of your blood vessels. Plasma is the liquid part of blood. Platelets are cells that help blood clot when blood vessels rupture from injury or damage.

Normally, platelets stop bleeding by sticking to damaged blood vessels and helping to form blood clots. Von Willebrand factor helps platelets stick. When you don’t have enough von Willebrand factor or you don’t have any factor, your platelets can’t stick as well as they should and it takes longer for platelets to help form blood clots.

Most people have von Willebrand disease because they inherited a mutated gene from one of their biological parents. This is autosomal dominant inheritance. Some people inherit mutated genes from both biological parents. This is autosomal recessive inheritance and is the most severe form of von Willebrand disease. People who carry the mutated gene have a 50% chance of passing the genetic mutation on to their biological children.

People also may develop von Willebrand disease as a complication of certain cancers, autoimmune disorders, heart and blood vessel diseases.

Diagnosis and Tests

How do healthcare providers diagnose von Willebrand disease?

Your healthcare provider will ask you to describe your symptoms. They may ask if you have biological family members who have similar symptoms or bleeding disorders. They may do the following tests:

  • Complete blood count (CBC): This test measures the numbers of your red blood cells, the different types of white blood cells and your platelets. It also measures the amount of hemoglobin in your red blood cells. Most people with von Willebrand disease have normal CBCs. People with unusually heavy bleeding may have low hemoglobin and red blood cell counts.
  • Platelet aggregation tests: Platelets are blood cells that help blood to clot. These tests measure how well your platelets stick together so they can help make blood clots.
  • Activated partial thromboplastin time test (APTT): Healthcare providers analyze other clotting factors, which, like von Willebrand factor, are proteins that help blood to clot. A lower than usual factor levels mean it takes longer for your blood to clot.
  • Prothrombin time (PT): This test measures additional clotting factors.
  • Fibrinogen test: Fibrinogen is another protein that helps blood to clot.
  • Von Willebrand factor antigen: This test measures the amount of von Willebrand factor protein in your bloodstream.
  • Ristocetin cofactor: This test evaluates von Willebrand factor activity.
  • Von Willebrand factor multimers: This test measures the factor’s structure.

Your healthcare provider may need to do several blood tests to confirm you have von Willebrand disease. That’s because factors, including hormone levels, may change your von Willebrand factor blood levels may change.

There’s more than one type of von Willebrand disease. Healthcare providers may do more laboratory tests to identify the specific defect. Here’s more information about von Willebrand disease types:

  • Type 1: This is the most common type of von Willebrand disease. It affects 60% to 80% of people with the disease. People with this type have low levels of von Willebrand factor in their blood. They may not have symptoms. If they have symptoms, those symptoms are mild.
  • Type 2: In this case, von Willebrand factor doesn’t work as it should. People with this type may have mild or moderate bleeding. About 15% to 30% of people with von Willebrand disease have this type.
  • Type 3: This is the most severe form of von Willebrand disease. It’s also the rarest type, affecting 5% to 10% of people with the disease. People with this type may have serious bleeding issues because they have very low von Willebrand factor levels or they don’t have any von Willebrand factor in their bloodstream.

Management and Treatment

How do healthcare providers treat von Willebrand disease?

Healthcare providers may treat this condition with different medications:

  • Desmopressin: This hormone boosts the levels of von Willebrand factor in your bloodstream. This is the most common treatment for von Willebrand disease.
  • Von Willebrand factor infusions: Some people may receive infusions of von Willebrand factor to stop bleeding episodes. They may receive this treatment before surgery. Some people with severe von Willebrand factor disease may receive regular infusions so they have a steady level of von Willebrand factor in their bloodstream.
  • Antifibrinolytics: These medications keep blood clots from breaking down. Your healthcare provider may prescribe this type of medication if you’re having dental surgery or if you’re someone who has heavy periods.
  • Birth control pills: This medication helps people with menstrual bleeding. It contains estrogen that increases von Willebrand factor levels in your bloodstream.
Care at Cleveland Clinic


Can I reduce my risk of having this disease?

Most people inherit von Willebrand disease. If your biological parents have this condition, you may inherit it from one or both of them.

Outlook / Prognosis

What can I expect if I have this condition?

Healthcare providers can treat von Willebrand disease but they can’t cure it. Most people have Type 1 or Type 2 von Willebrand disease and may only need treatment if they’re injured or need surgery. People with Type 3 von Willebrand disease may need ongoing medical treatment to manage bleeding.

Living With

I have von Willebrand disease. How do I take care of myself?

Most people with von Willebrand disease have mild or moderate symptoms. For them, living with von Willebrand disease may mean they should:

  • Avoid activities where they may get hurt, like contact sports such as football, rugby or hockey.
  • Tell all healthcare providers, including their dentists, that they have the disease. That way, their healthcare providers can plan how to manage bleeding after surgery or dental surgery.
  • Avoid aspirin and drugs that contain aspirin.
  • Avoid nonsteroidal anti-inflammatory (NSAIDs) like ibuprofen, unless a healthcare provider who knows they have von Willebrand disease tells them to take an NSAID.
  • Avoid nutritional supplement pills that contain vitamin E, fish oil or turmeric.
  • Consider medical alert identification. Wearing a medical alert bracelet or carrying identification may help people get appropriate medical care in an emergency.

When should I go to the emergency room?

Go to the emergency room any time you have bleeding you can’t control.

What questions should I ask my healthcare provider?

If you have von Willebrand disease, you may wonder how the disease will affect your life. Here are some questions you may want to ask your healthcare provider:

  • Why do I have this disease?
  • Can I pass this disease on to my children?
  • Will it get worse?
  • What are treatments?
  • What are treatment side effects?
  • Will this disease keep me from doing certain activities, like traveling or playing sports?

A note from Cleveland Clinic

Von Willebrand disease is a very common genetic blood disorder. Most people with this disease have mild or moderate symptoms. They may have frequent bloody noses or cuts that take a long time to stop bleeding. Other people have serious symptoms. For example, they may have joint pain that happens when blood flows into their joints and tissues. Some people may have the disease for years before they’re diagnosed. If you have this disease, you may be relieved to know why you have bleeding problems. You may worry your children may have this disease. Healthcare providers can’t cure this disease, but they can treat it. They’ll also answer your questions about passing it along to your children. And they’ll help you with information on living with von Willebrand disease so it doesn’t keep you from having an active, normal life.

Last reviewed by a Cleveland Clinic medical professional on 06/22/2022.


  • American Society of Hematology. 2021 Guidelines on the Diagnosis of von Willebrand Disease. (https://ashpublications.org/bloodadvances/article/5/1/280/474888/ASH-ISTH-NHF-WFH-2021-guidelines-on-the-diagnosis) Blood Adv. (2021) 5 (1): 280-300. Accessed 6/22/2022.
  • Canadian Hemophilia Society. Precautions in von Willebrand Disease. (https://www.hemophilia.ca/precautions-in-von-willebrand-disease/) Accessed 6/22/2022.
  • Fogarty H, Doherty D, O’Donnell J. New Developments in von Willebrand Disease. (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7658042/) Br J Haematol. 2020 Nov: 191(3): 329-339. Accessed 6/22/2022.
  • Merck Manuals. Von Willebrand Disease. (https://www.merckmanuals.com/home/blood-disorders/platelet-disorders/von-willebrand-disease?query=von%2520willebrand%2520disease) Accessed 6/22/2022.
  • National Heart, Lung and Blood Institute. What are Bleeding Disorders? (https://www.nhlbi.nih.gov/health/bleeding-disorders) Accessed 6/22/2022.

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