Symptoms
Von Willebrand factor helps blood cells stick together (clot) when you bleed. If there's not enough of it or it does not work properly, it takes longer for bleeding to stop. There's currently no cure for VWD, but it does not usually cause serious problems and most people with it can live normal, active lives.
Causes
Von Willebrand Disease vs Hemophilia
- Type 1 – this is an autosomal dominant variety of VW disease. There is a partial quantitative deficiency of the VWF
- Type 2 – type VW disease is also an autosomal dominant disorder characterized by a qualitative abnormality of the VWF
- Type 3 – there is a recessive inheritance which is associated with an almost complete deficiency of VWF.
Prevention
The recent American Society of Hematology/ISTH/National Hemophilia Foundation/World Federation of Hemophilia 2021 guidelines on the diagnosis of von Willebrand disease (VWD) is an outstanding effort to unify the diagnosis of VWD. However, as mentioned in ...
Complications
Von Willebrand disease is a lifelong bleeding disorder in which your blood doesn't clot properly. People with the disease have low levels of von Willebrand factor, a protein that helps blood clot, or the protein doesn't perform as it should.
Does von Willebrand disease have a cure?
What is the difference between von williebrand and hemophilia?
What is the prognosis for von Willebrand disease?
Does von Willebrand disease affect blood clotting?
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How do you get von Willebrand disease?
VWD is caused by genetic changes that are almost always inherited (passed down) from a parent to a child. A person's genes provide instructions on how to make proteins, such as the von Willebrand factor (VWF) protein. Each person has two copies of most genes, one from his or her mother and one from his or her father.
What is the main source of von Willebrand factor?
Von Willebrand factor is made within endothelial cells, which line the inside surface of blood vessels, and bone marrow cells. The factor is made of several identical subunits. To facilitate binding to various cells and proteins, these subunits are cut into smaller pieces by an enzyme called ADAMTS13.
Who is most likely to get von Willebrand?
Prevalence. Von Willebrand disease (VWD) occurs with equal frequency among men and women, affecting up to 1% of the general population. However, women are more likely to experience symptoms of VWD because of the increased bleeding it causes during their menstrual periods, during pregnancy, and after childbirth.
Is von Willebrand disease acquired or inherited?
Inherited von Willebrand disease (VWD) is the most common inherited bleeding disorder.
Is von Willebrand an autoimmune disease?
While the more common types of von Willebrand's disease are inherited, it is also possible to develop the condition from an autoimmune disease such as lupus or cancer, or as a result of taking some medications. This is called acquired von Willebrand's disease.
Can you outgrow von Willebrand disease?
Von Willebrand disease can't be cured. But with treatment and self-care, most people with this disease can lead active lives.
What foods should be avoided with von Willebrand disease?
Food and supplements to avoidlarge glasses of juice.soft drinks, energy drinks, and sweetened tea.heavy gravies and sauces.butter, shortening, or lard.full-fat dairy products.candy.foods containing trans fats, including fried. foods and baked goods (pastries, pizza, pie, cookies, and crackers)
Does von Willebrand make you tired?
The weakening, fatigue and other disruptive effects of VWD can be stopped. Even childbirth can be made safe for a mother and baby. If you have unexplained bleeding symptoms, remember to ask your doctor, “Could I have von Willebrand disease?”
Can you get the Covid vaccine if you have von Willebrand?
Patients with von Willebrand disease or a rare bleeding disorder should consult with their hematologist regarding special precautions prior to receiving the vaccination. All rare bleeding disorder patients (including those with thrombocytopenia and/or platelet function disorders) should be vaccinated.
Is von Willebrand disease considered a disability?
The condition is medically equal to a condition in the Listing of Impairments. If someone can prove that his or her von Willebrand disease is medically equal to any listing in the Listing of Impairments then that person may qualify for Social Security disability.
What are the 3 types of von Willebrand disease?
Types of von Willebrand diseasetype 1 – the mildest and most common type. People with type 1 VWD have a reduced level of von Willebrand factor in their blood. ... type 2 – in people with this type of VWD, von Willebrand factor does not work properly. ... type 3 – the most severe and rarest type.
Can I donate blood with von Willebrand disease?
Simply put, anyone with a bleeding disorder or symptoms of a bleeding disorder should not donate blood.
What is von Willebrand disease?
Von Willebrand disease (VWD) is a bleeding disorder in which your blood has trouble clotting. It is inherited, meaning it is passed down from parents to their children. People with von Willebrand disease sometimes experience heavier-than-normal bleeding from injury, surgery, and, in women, menstrual flow and childbirth.
Can von Willebrand cause bleeding?
Abnormal bleeding can cause problems in menstrual cycles and after childbirth. People with severe von Willebrand disease (Type 3) may experience severe pain and swelling if bleeding occurs in their joints or soft tissues. In extreme cases, without treatment, excessive bleeding can lead to death.
What is von Willebrand disease?
Von Willebrand disease is a bleeding disorder. It’s caused by a deficiency of von Willebrand factor (VWF). This is a type of protein that helps your blood to clot. Von Willebrand is different from hemophilia, another type of bleeding disorder. Bleeding happens when one of your blood vessels breaks.
How much of the population is affected by von Willebrand disease?
This leads to prolonged bleeding. According to the Centers for Disease Control and Prevention. Trusted Source. , von Willebrand disease affects up to 1 percent of the general population in the United States.
What is the drug used for VWF?
Nonreplacement therapy. Your doctor may prescribe desmopressin (DDAVP), which is a drug recommended for types 1 and 2A. DDAVP stimulates the release of VWF from your body’s cells. Common side effects include a headache, low blood pressure, and fast heart rate.
What is the most dangerous type of von Willebrand disease?
Type 3 is the most dangerous type of von Willebrand disease. If you have this type, your body won’t produce any VWF. As a result, your platelets won’t be able to clot. This will put you at risk of severe bleeding that’s difficult to stop.
What happens when blood vessels break?
Bleeding happens when one of your blood vessels breaks. Platelets are a type of cell that circulates in your blood and clumps together to plug broken blood vessels and stop bleeding . VWF is a protein that helps platelets clump together, or clot.
What drugs should I avoid for Von Willebrand?
If you have any form of Von Willebrand disease, it’s important to avoid drugs that can increase your risk of bleeding and complications. For example, avoid aspirin and nonsteroidal anti-inflammatory drugs, such as ibuprofen and naproxen.
Can you get Von Willebrand disease if you have a mutated gene?
For example, you can only develop type 3 Von Willebrand if you’ve inherited a mutated gene from both of your parents . If you’ve only inherited one copy of the mutated gene, you’ll develop type 1 or 2 von Willebrand disease.
What is von Willebrand disease?
Related Pages. Von Willebrand disease (VWD) is a blood disorder in which the blood does not clot properly. Blood contains many proteins that help the blood clot when needed. One of these proteins is called von Willebrand factor (VWF). People with VWD either have a low level of VWF in their blood or the VWF protein doesn’t work the way it should.
What type of blood clotting protein is most common in VWD?
Type 1. This is the most common and mildest form of VWD, in which a person has lower-than-normal levels of VWF. A person with Type 1 VWD also might have low levels of factor VIII (8), another type of blood-clotting protein. About 85% of people treated for VWD have Type 1.
How long does VWD bleeding last?
This bleeding may be characterized in the following ways: After a cut to the skin, the bleeding lasts more than 5 minutes.
Why is VWF important?
Because the treatment is different for each type, it’s important that a person know which subtype he or she has. In Type 2A, the VWF is not the right size and doesn’t help the platelets attach together in order to form a clot. In Type 2B, the VWF attaches to platelets at the wrong time (when there is no injury).
How many people in the US have VWD?
This means that 3.2 million (or about 1 in every 100) people in the United States have the disease. Although VWD occurs among men and women equally, women are more likely to notice the symptoms because of heavy or abnormal bleeding during their menstrual periods and after childbirth.
What is VWD type 2?
Type 2. With this type of VWD, although the body makes normal amounts of the VWF, the factor does not work the way it should. Type 2 is further broken down into four subtypes―2A, 2B, 2M, and 2N―depending on the specific problem with the person’s VWF.
Does VWF work in blood?
People with VWD either have a low level of VWF in their blood or the VWF protein doesn’t work the way it should. Normally, when a person is injured and starts to bleed, the VWF in the blood attaches to small blood cells called platelets.
Why is genetic testing not used?
Genetic testing is not frequently used for those with type 1 von Willebrand disease due to several factors, including cost, technical complexity of the testing due to the nature of the von Willebrand factor gene, and lack of usefulness of the information that is currently provided by the results. As more knowledge is gained about ...
Is von Willebrand disease a bleeding disorder?
Though von Willebrand disease is a common condition, genetic testing is not performed for it as frequently as it is for many other bleeding disorders. Genetic testing of the von Willebrand factor gene is most useful in determining the exact subtype for those with type 2 von Willebrand disease, or to differentiate between type 2N von Willebrand disease and mild factor VIII deficiency (hemophilia A).
What is von Willebrand disease?
Listen. Von Willebrand disease is typically an inherited disease caused by variations ( mutations) in the VWF gene. The VWF gene provides instructions for making a blood clotting protein called von Willebrand factor, which is important for forming blood clots and preventing further blood loss after an injury.
What causes von Willebrand factor to rise?
Increased age, pregnancy, exercise, and stress may cause von Willebrand factor levels in the blood to rise, which can make bleeding symptoms less frequent. This disease is caused by mutations in the VWF gene and can have different inheritance patterns. [1] .
What is AVWS in medical terms?
Another rare and different condition called acquired von Willebrand syndrome (AVWS). is a mild to moderate bleeding disorder that is typically seen in conjunction with other disorders, such as diseases that affect bone marrow or immune cell function.
When was von Willebrand factor approved?
Recombinant von Willebrand factor (rhVWF) (Brand name: Vonvendi) - Manufactured by Baxalta US, Inc. FDA-approved indication: April 2018 approved for use in adults (age 18 and older) diagnosed with von Willebrand disease (VWD) for perioperative management of bleeding.
Is von Willebrand disease inherited?
Most, but not all, cases of von Willebrand disease (VWD) are inherited. The majority of cases of type 1 and type 2A, as well as type 2B and type 2M, are inherited in an autosomal dominant manner. VWD type 2N, type 3, and some cases of type 1 and type 2A are inherited in an autosomal recessive manner. [5]
What is Von Willebrand's disease?
Risk factors and causes. Diagnosis. Treatment. Complications. Preventing bleeding episodes. Summary. Von Willebrand’s disease is a hereditary blood-clotting disorder. It is the most common bleeding condition that a person can inherit. However, a person might also acquire it as a result of other medical conditions, such as lymphomas, leukemias, ...
How many genes are needed for von Willebrand's disease?
For example, in types 1 and 2, only one gene is necessary to cause the condition. Also, the biological parents will likely have von Willebrand’s disease themselves. In type 3, both parents need to pass on genes, and they will most likely be carrying the disease without actually having it.
What is the most common type of bleeding disorder?
Von Willebrand’s disease is the most common type of heritable bleeding disorder. Type 1 is the most prevalent, affecting around 60–80 percent of all people with the condition. Symptoms may be mild or severe, and they may include nosebleeds, excessive bleeding from a cut, and bruising more easily.
What is the physical exam for von Willebrand's disease?
The physical exam will check for bruising and signs of recent bleeding. Blood tests can assess: vWF levels. the structure of vWF and its multimers, or protein complexes, as well as how its molecules break down, to determine the type of von Willebrand’s disease.
What causes type 2?
Different gene mutations can cause type 2, and each will require different treatment. Knowing the type 2 subtype can help a doctor shape treatment in a way that works best for the individual. Type 2 is present in around 15–30 percent of people who have von Willebrand’s disease. Type 3.
Does von Willebrand's disease cause clots?
People with von Willebrand’s disease either lack, or have ineffective supplies of, a substance in the blood called von Willebrand factor (vWF). This promotes clotting. People with the condition will have difficulty forming blood clots. For example, if they cut themselves, it will take longer to stop bleeding.
Is clotting factor VIII faulty?
Clotting factor VIII is either missing or faulty in people with the most common form of hemophilia. Family history is the most common risk factor for von Willebrand’s disease. That said, the genetic content needed for each type to develop will differ.
Causes
Epidemiology
Diagnosis
Risks
Symptoms
Treatment
- 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 togethe…
Contraindications
Side effects
Overview
Prevention
- Von Willebrand disease is a bleeding disorder. Its caused by a deficiency of von Willebrand factor (VWF). This is a type of protein that helps your blood to clot. Von Willebrand is different from hemophilia, another type of bleeding disorder. Bleeding happens when one of your blood vessels breaks. Platelets are a type of cell that circulates in you...
Prognosis
- According to the Centers for Disease Control and Prevention, von Willebrand disease affects up to 1 percent of the general population in the United States. Men and women develop von Willebrand disease at the same rate. But women are more likely to experience symptoms and complications due to the increased risk of bleeding during menstruation, pregnancy, and childbirth.
Who Is Affected?
- If you have type 2 von Willebrand disease, you have normal levels of VWF but it wont work properly due to structural and functional defects. Type 2 is divided into subtypes, including types: Your doctor will ask you questions about your personal and family history of abnormal bruising and bleeding. Type 3 tends to be the easiest to diagnose. If you have it, youll likely have a histor…
Types of VWD
- Type 3 is the most dangerous type of von Willebrand disease. If you have this type, your body wont produce any VWF. As a result, your platelets wont be able to clot. This will put you at risk of severe bleeding thats difficult to stop.
Causes
- If you have von Willebrand disease, your symptoms will vary depending on which type of the disease you have. The most common symptoms that occur in all three types include: Type 3 is the most severe form of the condition. If you have this type, you have no VWF in your body. This makes episodes of bleeding difficult to control. It also raises your risk of internal bleeding, inclu…
Signs and Symptoms
- If you have von Willebrand disease, your treatment plan will vary, depending on the type of condition you have. Your doctor may recommend several different treatments. Your doctor may prescribe desmopressin (DDAVP), which is a drug recommended for types 1 and 2A. DDAVP stimulates the release of VWF from your bodys cells. Common side effects include a headache, …
Diagnosis
- These replacement therapies arent identical and you shouldnt use them interchangeably. Your doctor may prescribe Humate-P if you have type 2 von Willebrand disease and are unable to tolerate DDAVP. They may also prescribe it if you have a severe case of type 3 von Willebrand disease.
Treatments
- Common side effects of replacement therapy with Humate-P and Alphanate SD/HT include chest tightness, rash, and swelling.