Willebrand Disease

Von Willebrand disease, or vWD, is an inherited disorder that affects the blood's ability to clot properly. As a group, bleeding disorders (including hemophilia) are rare. Von Willebrand disease affects as much as 1% of the population or more.
A substance in the blood known as von Willebrand factor helps platelets stick to damaged blood vessels. Another function of von Willebrand factor is to carry the important clotting protein, called factor VIII, in the blood. People with vWD have a problem with one or both of these blood components.

In many people with von Willebrand disease (vWD), the signs are mild or they may be absent altogether. When signs occur, their intensity can vary from one person to another. Von Willebrand disease is often challenging to diagnose in milder cases.

Symptoms

Abnormal bleeding is the most common sign of von Willebrand disease, although it may be present at only moderate levels.

The abnormal bleeding associated with von Willebrand disease may occur as:

* Recurrent and prolonged nosebleeds
* Bleeding from the gums
* Increased menstrual flow
* Excessive bleeding from a cut or following a tooth extraction or other dental procedure
* Blood in the stool or urine
* Bleeding from shaving with a razor
* Easy bruising
* Bruises with lumps that form underneath the skin

Some people may realize that they have a bleeding disorder only after a surgical procedure or serious trauma in which excessive bleeding occurs.

Diagnosis

Ideally, a simple, single laboratory test could screen for the presence of VWD. Such a screening test would need to be sensitive to the presence of most types of VWD and would have a low false—positive rate. Unfortunately, no such test is available. In the past, the activated partial thromboplastin time (PTT) and bleeding time (BT) were recommended as diagnostic tests.

Treatment

Mild bleeds may be treated with pressure and are often self-limiting.

Hormones found in oral contraceptives administered intravenously, orally, topically, or by patch application.

Localized bleeding in the nose or mouth may be treated at the site with Thrombinar, Avitene, or Instat followed by a medication such as Amicar.



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