Von Willebrand disease is a common, hereditary, blood-clotting disorder.

It is the most common inherited bleeding condition, but it can also be acquired from other medical conditions, such as lymphomas and leukemias, autoimmune disorders, such as lupus, and certain medications.

A person with von Willebrand Disease (vWD) lack von Willebrand Factor (vWF), so that they have difficulty forming blood clots. It will take them longer than other people for bleeding to stop after a cut.

As in hemophilia, clotting factor VIII is either missing or faulty in patients with vWD. VWD is more common than hemophilia, but it is usually milder.

It affects up to 1 percent of people in the United States.

There is no cure, but treatment can enable patients to lead normal and healthy lives.

Types

There are three main types of vWD:

Type 1 von Willebrand disease


Symptoms of vWF include bruising.

Type 1 is the most common type of inherited von Willibrand disease. Around 60 percent to 80 percent of cases are type 1.

In type 1, the patient has low von Willebrand factor and low factor VIII, a clotting factor.

When only factor VIII is low, it is called hemophilia A.

Type 1 can range in severity from mild to severity, but mostly, it is mild.

Type 2 vWD

There are several subtypes, but Type 2 vWD is when the vWF does not work properly. Type 2 is caused by different gene mutations, and they receive different medical treatment. It is important to know what subtype of vWD type 2 the patient has. Type 2 accounts for 15 percent to 30 percent of cases.

Type 3 vWD

The patient typically has low levels of factor VIII and no vWF. This is the rarest and most severe form of vWD, and it affects 5 percent to 10 percent of people with the condition.

Acquire vWD develops as the result of an autoimmune disease, such as lupus or cancer.

Symptoms

Signs and symptoms may be mild and go unnoticed, or they may appear in varying ways. Doctors commonly do not spot milder forms of vWD.

When symptoms appear, they include bruising and extended or excessive bleeding. There may be bleeding from the mucous membranes, including the gastrointestinal tract.

Symptoms of bleeding include:

  • Nosebleeds, which may be prolonged, recurring, or both
  • Bleeding from the gums
  • Longer, heavier menstrual bleeding In females
  • Excessive bleeding from a cut
  • Excessive bleeding after a tooth extraction or other dental work
  • Bruising, sometimes with lumps forming under the skin.

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Sometimes, vWF is only discovered after a surgical procedure, dental work, or serious trauma.

Women may notice the following signs related to menstruation:

  • Blood clots at least 2.5 centimeters (1inch) in diameter
  • Soaking through two or more tampons/pads in two hours
  • Need for double sanitary protection to control bleeding
  • Menstruation continues for over a week
  • Signs and symptoms of anemia, including fatigue, panting, and drowsiness.

Rarely, the bleeding can damage internal organs. In this case, it can be fatal.

Risk factors and causes

When a blood vessel is injured, small blood cell fragments called platelets normally clump together to plug the hole in the blood vessel and stem the bleeding.


In people with von Willebrand Disease, blood does not clot as it should.

VWF helps platelets to stick together and form a blood clot. VWF also carries clotting factor VIII, a vital protein for blood clotting. Clotting factor VIII is either missing or faulty in patients with hemophilia and other clotting disorders.

A person with vWD does not have enough vWF, or their vWF does not work properly.

Family history is the most common risk factor for vWD. A person with the gene has a 50 percent chance of passing it on. If both parents have the defective gene, their children risk having vWD type 3, the severest and rarest form.

Acquired vWD, resulting from another condition, can happen later in life.

Diagnosis

Early diagnosis and treatment significantly increase the chances of living a normal and active life. Some patients with type 1 or type 2 may not have major bleeding problems, so they may not be diagnosed until they experience surgery or a serious injury.

Diagnosis of vWD type 3 usually happens early in life, because major bleeding will probably occur at some time during infancy and childhood.

The physician will look at the patient’s medical history, carry out a physical exam, and request some diagnostic tests.

When assessing medical history, the doctor may ask whether the patient has ever had:

  • Excessive bleeding after surgery or a dental procedure
  • Unexpected or easy bruising, or bruising with a lump underneath
  • Unexpected blood in the feces
  • Bleeding of the joints or muscles
  • Bleeding after using medications, such as aspirin, non-steroidal anti-inflammatory drugs (NSAIDs), or blood thinners
  • Abnormal blood platelet counts
  • Unexplainable nosebleeds lasting over 10 minutes, despite placing pressure on the nose
  • Frequent nosebleeds
  • Heavy menstrual bleeding, with clots, for longer than a week
  • A history of kidney, liver, blood, or bone marrow disease.

The physical exam will check for bruising and signs of recent bleeding.

Blood tests can assess:

  • Levels of vWF antigen, or protein
  • Ristocetin cofactor activity, to reveal how well vWF works, and whether it is adequately clotting the blood
  • Factor VIII clotting activity, to establish levels of factor VIII
  • The structure of Von Willebrand factor (vWF) and its multimers, or protein complexes, and how its molecules break down, to determine the type of vWD involved
  • Platelet function
  • Bleeding time, to see how long it takes for a small wound to stop bleeding.

Test results may take 2 to 3 weeks, and some tests may need repeating, to confirm a diagnosis. The patient may be referred to a hematologist, a doctor specialized in conditions of the blood.

Treatment

There is no cure for vWD, but treatment, mainly medication, is available to prevent or stop bleeding episodes.

Symptoms of vWD are normally mild, and treatment is only needed during surgery, dental work, or after an accident or injury. Treatment depends on the type and severity of the condition, and how the patient responds to therapy.

Medications can:

  • Control heavy menstruation
  • Release more vWF and factor VIII into the bloodstream
  • Prevent the breakdown of blood clots
  • Replace missing vWF.

Desmopressin (DDAVP) is a synthetic hormone given by injection or through a nasal spray (Stimate). It is similar to vasopressin, a natural hormone that controls bleeding by making the body release more of the vWF that is already stored in the lining of the blood vessels. This, in turn, raises factor VIII levels. It is usually prescribed for individuals with vWD types 1 and 2.

The nasal spray may be used at the beginning of a menstrual period, or by doctors before minor surgery.

If DDAVP is not sufficient, doses of concentrated blood clotting factors containing vWF and factor VIII may be prescribed for all three types of vWD.

Oral contraceptives, specifically birth control pills containing estrogen, can help women with heavy periods.

Clot-stabilizing, or antifibrinolytic, medications delay the breakdown of clotting factors. This helps to keep a clot in place once it is formed, especially around the time of surgery or dental work.

Fibrin sealants are a glue-like substance that is applied directly onto a wound to stop bleeding.

Complications

Levels of vWF and factor VIII normally increase during pregnancy, but bleeding complications can occur during delivery, and bleeding may be heavy and last longer after giving birth.

A woman with vWD should consult a hematologist and specialized obstetrician on becoming pregnant. She will probably have blood tests during the last trimester.

Prevention of bleeding episodes

To prevent bleeding episodes, people with vWD should check with a physician before taking medications, and they should avoid over-the-counter (OTC) medications that may affect blood clotting, such as aspirin, ibuprofen, and other NSAIDs.

They should also inform health care professionals, including dentists, and sports coaches about their condition. People with severe symptoms should wear a medical ID necklace or bracelet.

To minimize common health risks, people with vWD should eat a healthy, balanced diet, and exercise regularly. Normally, vWD does not interfere with daily activities, but children may be advised to avoid contact sports, like football and hockey.

Source: What is Von Willebrand disease?

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