von Willebrand Disease
What is von Willebrand Disease?
The most common bleeding disorder is von Willebrand disease (VWD) which affects 1% of the world’s population. VWD is typically an inherited disease and caused by deficient or defective von Willebrand Factor (VWF). It occurs in both men and women and is diagnosed by measuring the level and activity of VWF and factor VIII.
VWD occurs when von Willebrand factor (VWF), a protein that helps form blood clots to stop bleeding, is missing or doesn’t work the way it should. Because of this, when people with VWD bleed from cuts or injuries, their bleeding takes longer to stop.
There are three main types of von Willebrand disease. Type 1 is most common and the mildest, and type 3 the rarest and most severe form. Type 3 is usually inherited in an autosomal recessive manner in which the child receives defective gene from both parents. People with type 3 condition have very little or no VWF and experience spontaneous bleeding episodes often into joints and muscles. People with VWD experience prolonged bleeding after injury, surgery, dental work or childbirth. Women with VWD have heavy or prolonged menstrual bleeding.
The treatment depends on the type and severity of VWD. Type 3 patients and patients at risk of severe bleeding in type 1 and type 2 are often treated with factor concentrates that contain FVIII and are rich in VWF.
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