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Von Willebrand Disease 2016-11-07T21:10:05+00:00

WHAT IS VON WILLEBRAND DISEASE?

A bleeding disorder in which von Willebrand factor (vWF), a blood protein, is either deficient or defective. It is the most common inherited bleeding disorder and is caused by a deficiency or defect of a blood clotting protein call von Willebrand Factor. Von Willebrand factor is a glue-like protein that interacts with platelets to form a plug which prevents the blood from flowing at the site injury. This is called a platelet plug. If a person does not have enough vWF, or if the factor does not work properly, no platelet plug will form and bleeding will continue for a longer period of time.

THREE MAJOR TYPES OF Von Willebrand Disease (vWD)

People with Type 1 vWD have low levels of vWF and may have low levels of factor VIII(8). Type is the most common form of vWD. This type is most often associated with mucosal bleeding.
In Type 2 vWD, the vWF does not work properly. Type 2 is divided into four different subtypes: 2A, 2B, 2M and 2N. Each sub-type has a different type of bleeding associated with it. Extra blood tests may be ordered to determine the sub-type. This helps the doctors know what medications work bet for the patient’s type of bleeding.
Type 3 vWD usually has no vWF and low levels of factor VIII(8). Type 3 is the most severe form of vWD. People with vWD can bleed into their joints and muscles or have more severe bleeding like someone with sever Hemophilia A or B.

SYMPTOMS

Although vWD occurs both in men and women, women are more likely to notice the symptoms because of abnormal bleeding during their menstrual period or after childbirth. The disorder can be inherited from either parent. It has been estimated that vWD affects up to one percent of the population. However, it is generally the least severe of the clotting disorders. Many people with vWD will have few, if any, symptoms. The main symptoms are easy bruising, frequent or prolonged nosebleeds, increased menstrual bleeding, and prolonged bleeding following injury, surgery, dental work, or childbirth.

TREATMENT

vWD is treated with desmopressin (also called DDAVP), amniocaproic acid, tranxemic acid, or with infusions of a clotting factor concentrate that contains vWF.