Acquired von Willebrand disease (AvWD) is a type of von Willebrand disease (vWD) that isn’t inherited. Instead, it can be due to other medical conditions or certain medications.

vWD is a condition of the blood that slows the clotting process. It’s usually inherited, but people can also develop it secondary to a different condition. This is called AvWD.

Both genetic and acquired vWD are uncommon. The genetic form affects fewer than 200,000 people in the United States, but estimates are as high as 1 in 100 because those with mild symptoms might not have diagnoses. AvWD is even more uncommon.

People typically get AvWD during adulthood. It’s most common among people with other medical conditions. It can also happen because of certain drugs.

Some conditions associated with AvWD are:

  • Lymphoproliferative disorders: These cause the overproduction of lymphocytes, a type of immune cell, which in turn causes the immune system to malfunction.
  • Autoimmune conditions: These cause the body’s immune system to attack healthy cells. Examples of conditions that might lead to AvWD include systemic lupus erythematosus, scleroderma, and antiphospholipid antibody syndrome.
  • Heart conditions: Some cardiac conditions, like aortic valvular stenosis, can lead to AvWD.
  • Essential thrombocythemia: This is when you have an increased number of platelets in your blood.

Types of vWD

There are four types of vWD. Types 1, 2, and 3 are inherited. AvWD is the fourth type.

  • Type 1: People with type 1 have less healthy von Willebrand factor (vWF) than people without vWD. It’s the mildest type, accounting for about 80% of genetic cases.
  • Type 2: Type 2 is divided into four subtypes according to differences in how platelets bind. In type 2, vWF doesn’t work properly.
  • Type 3: In type 3, there’s no vWF at all. This is the most severe type. About 5% of genetic cases are type 3.
  • Acquired: AvWD is any condition of vWF that’s not inherited.

vWD has a number of symptoms, all of which indicate that there might be an issue with how blood clots. Those symptoms include:

  • bleeding from cuts, injuries, or nosebleeds lasting longer than 10 minutes
  • raised large bruises and easy bruising
  • anemia or low iron levels
  • heavy bleeding after surgery
  • heavy menstrual bleeding
  • heavy bleeding after childbirth or miscarriage
  • gastrointestinal hemorrhage along with anemia

For cases of genetic vWD, a person might have a family history of a bleeding condition or vWD symptoms.

vWF is a type of cellular protein that’s critical to blood clotting. It forms a bridge between platelets and the lining of blood vessels. When vWF doesn’t work properly, this bridging is slow to occur, and blood is slow to clot.

In genetic vWD, vWF doesn’t work properly because of an inherited genetic difference. In type 3 vWD, vWF is missing completely. In AvWD, the problems with vWF function are because of another condition, like an autoimmune condition.

With the acquired form of the condition, vWF works effectively to support blood clotting until the secondary condition disrupts its normal function. How this happens depends on the underlying condition. One example is myeloproliferative disorders, which are types of blood cancers. They cause the immune system to develop antibodies against vWF.

Doctors diagnose genetic vWD based on the amount of vWF in the blood, though no one test is conclusive. Typically, doctors don’t test if there’s no personal history of bleeding or no family history of vWD or a bleeding condition. There are detailed guidelines about how to test for specific types of genetic vWD.

There’s no specific diagnostic procedure for AvWD. Doctors diagnose it through laboratory findings and symptoms that look like the genetic form of vWD.

If a person has gastrointestinal bleeding, a doctor might use endoscopy to examine the gastrointestinal tract to search for other causes, like polyps, ulcers, or cancer. If there’s no other cause, they might look to a malfunction of vWF as the cause.

The only way to cure AvWD is to resolve the underlying medical condition that causes it. So, the treatment strategy is to focus on the autoimmune condition, cancer, or other diagnosis that’s leading to the AvWD. The nature of that treatment depends on the condition.

The other objectives of AvWD treatment are to stop acute bleeding and to prevent bleeding in high risk situations. Doctors use a combination of medications that can increase blood clotting time to achieve these objectives.

It’s sometimes possible to cure AvWD if you effectively treat the underlying condition. Otherwise, you’ll stay at a high risk of bleeding, especially in high risk situations like surgery.

AvWD is a rare blood clotting condition due to another medical condition. It disrupts the normal activity of vWF in the blood.

Resolving the underlying condition can cure AvWD. Otherwise, the goal is to reduce acute bleeding and lower the risk of bleeding in high risk situations.