Von Willebrand Disease

Written by Lydia Krause
Medically Reviewed by George Krucik, MD

What Is Von Willebrand Disease?

Von Willebrand disease is a bleeding disorder caused by a deficiency of von Willebrand factor (VWF).

To understand this disease, you need to understand a little bit about how blood clotting works. Platelets are circulating blood cells that have the job of clumping together and plugging broken blood vessels to stop bleeding. VWF is responsible for helping platelets clump (clot) together. If VWF is low or absent in your body, the platelets cannot clump properly. This leads to prolonged bleeding.

Von Willebrand disease is fairly rare. According to the Centers for Disease Control and Prevention, the disease affects (at most) 1 percent of the general population (CDC, 2011).

Types of Von Willebrand Disease

There are three types of von Willebrand disease.

Type 1

This is the most common type. In this type, there are small amounts of VWF present in the body to help clot blood. Most individuals with type 1 von Willebrand disease have mild bleeding and are able to live a normal life.

Type 2

Individuals with type 2 von Willebrand disease have normal levels of VWF, but there are defects in the factor’s function and structure. Type 2 is divided into subtypes depending on severity. These include 2A, 2B, 2M, and 2N.

Type 3

This is the most dangerous type because there is no VWF at all present in the body, so platelets cannot clot. Bleeding in individuals with type 3 von Willebrand disease is severe and difficult to stop.

What Causes Von Willebrand Disease?

Von Willebrand disease is caused by a genetic mutation. All of the body’s characteristics (eye color, height, hair color, etc.) are determined by the body’s DNA. Sometimes, abnormal changes called “mutations” can occur to your DNA. Mutations can sometimes be harmless, but in other cases, can cause diseases like Von Willebrand.

The severity of this disorder depends upon whether one or both parents carried the mutation and passed it to their offspring. For example, a person can only develop type 3 Von Willebrand if he or she has inherited a genetic mutation from both parents. If a person only has one parent with a genetic mutation, he or she will only have type 1 or 2.

Symptoms of Von Willebrand Disease

Bleeding symptoms and their severity vary depending on the type of von Willebrand disease. The most common symptoms of all three types include:

  • excessive nose bleeds
  • abnormally heavy bleeding during menstruation
  • bleeding from the gums
  • easy bruising

Type 3, however, is the most severe. In this type, there is no VWF in the body, so bleeding can be extremely difficult to control. Rare complications also seen in type 3 include gastrointestinal and internal bleeding (especially in the joints).

Diagnosis of Von Willebrand Disease

Diagnosis of von Willebrand disease focuses on a family and personal history of abnormal bleeding. Those with type 3 are the easiest to diagnose, as these individuals began life with severe bleeding. Along with history, doctors may also use different laboratory tests to look for low or absent VWF and to measure plasma levels as well as the time it takes for bleeding to stop. Because of their specialized nature, it may take two to three weeks to receive blood test results (Mayo Clinic, 2011).

Treatment for Von Willebrand Disease

Treatment for von Willebrand disease is tailored to the type and severity of the disorder. There are a number of different therapies for treating this disease.

Nonreplacement Therapy

Desmopressin (DDVAP) is the drug recommended for types 1 and 2A. DDVAP stimulates the release of VWF from the body’s cells. Common side effects include headache, low blood pressure, and a fast heart rate.

Replacement Therapy

Humate-P and Alphanate SD/HT are created from plasma to replace the body’s VWF. These replacement therapies are not identical, so they should never be interchanged. Humate-P is for those unable to tolerate DDVAP and is used for any type 2 or severe type 3 cases. Common side effects of Humate-P and Alphanate SD/HT include chest tightness, rash, and swelling.

Other Therapies

Aminocaproic acid and tranexamic are both drugs that steady the clots formed by platelets. These drugs are used before invasive surgeries or to treat individuals with type 1 von Willebrand. Common side effects include nausea, vomiting, and clot complications.

Topical Options

In the case of minor bleeding from small capillaries or veins, the topical agent Thrombin-JMI can be applied. Tisseel VH is topically applied after surgery, but will not stop heavy bleeding.

Drugs to Avoid

If you any form of Von Willebrand, avoid aspirin and nonsteroidal anti-inflammatory drugs (NSAIDs). Both of these drugs can increase bleeding complications.

Long-Term Outlook for Those With Von Willebrand Disease

Most patients with type 1 von Willebrand disease are able to live normal lives with only mild bleeding issues.

Those with type 2 are at greater risk for complications and experience mild to moderate bleeding. These individuals may suffer worse bleeding in the case of infection, surgery, or pregnancy.

Individuals with type 3 are at risk for severe bleeding as well as internal and gastrointestinal bleeding.

In all cases, people living with von Willebrand must take caution to warn health providers, including their dentists, of their condition. Your family and close friends should also know of your condition in case of an unexpected accident and/or the need for life-saving surgery.

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