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Von Willebrand's Disorder

Von Willebrand's disorder is caused by a deficiency (type 1) or an abnormality (type 2) of von Willebrand factor. It is the commonest of the inherited bleeding disorders, occurring in about 1 in 1000 of the population. Occasionally a severe disorder, von Willebrand's disorder (type 3) can occur. It has similar symptoms to Haemophilia.

Von Willebrand's disorder differs in a number of respects from haemophilia.

It is usually mild.
It occurs in both males and females.
It can be passed on both by males and females.
Levels of von Willebrand factor vary from day to day, due to stress, exercise and hormones, sometimes making diagnosis difficult.

Joint bleeds are uncommon and most people experience excessive bruising, bleeding from mucous membranes such as nose bleeds and bleeding following some types of surgery or dental extractions. In women, heavy periods may be experienced.

Treatment for von Willebrand's disorder varies depending on the type (1,2,or 3) and the severity based on symptoms and results of blood tests.
In those with Type1 von Willebrand's disorder and mild reductions in von Willebrand factor, a drug DDAVP (desmopressin acetate) is given intravenously to stimulate the release of von Willebrand factor into the circulation and prevents or treats bleeding. This infusion may need to be repeated. Intravenous Factor V111 concentrate can be used in cases where DDAVP is not effective. Tranexamic acid tablets are often helpful in women who are experiencing heavy periods..

This page last updated: November 2004
Review date: June 2005

QLD Haemophilia Centre

Von Willebrand's Disorder