What Is Von Willebrand Disease?

Von Willebrand disease, also known as vWD, is the most common hereditary blood clotting (coagulation) abnormality described in humans - in other words, it is the most common inherited bleeding condition. Von Willebrand disease, however, can also result from other medical conditions. The patient suffers from extended or excessive bleeding. The bleeding may sometimes damage internal organs, and even cause death, although this is rare.

VWD may arise from a quantitative or qualitative deficiency of vWF (von Willebrand factor), a multimeric protein that is required for platelet adhesion - i.e., patients either have low levels of that protein in their blood, or the protein does not work properly. VWF is a vital component in the blood clotting process. Patients take longer to form blood clots; when they are cut it takes longer for them to stop bleeding.

When a blood vessel is injured, small blood cell fragments called platelets will normally clump together to plug the hole in the blood vessel and stem the bleeding. VWF is a glue-like substance that helps the platelets stick together and form a blood clot. VWF also carries clotting factor VIII, a vital protein in the blood clotting process. Clotting factor VIII is either missing or faulty in patients who have hemophilia, or some other clotting disorder.

Although von Willebrand disease is much more common than hemophilia, it is usually milder.

According to the NIH (National Institutes of Health), USA, 1 in every 100 to 1,000 people in the USA is affected. While hemophilia affects males, VWD affects both sexes.

The condition is known to affect humans and dogs (especially Doberman Pinschers), and in very rare cases swine, cattle, horses and cats.

Other factors, including ABO blood groups may also influence the severity of the condition.

Von Willebrand disease treatment focuses on preventing or stopping bleeding episodes, mainly with medications. With proper treatment patients can lead normal and healthy lives.

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