What is Von Willebrand disease?

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

[bruise]
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.

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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.

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