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Von Willebrand disease
Von Willebrand's disease (vWD) is a hereditary coagulation abnormality
that arises from a deficiency of von Willebrand factor (vWF), a
substance that affects platelet adhesion. It is known to affect humans
and, in veterinarian medicine, dogs.
History
vWD is named after Erik Adolf von Willebrand, a Finnish internist
(1870-1949). He first described the disease in 1926.
Epidemiology
In humans, the incidence of vWD is roughly about 1 in 1000 individuals.
Because most forms are rather mild, they are detected more often in
women, whose bleeding tendency shows during menstruation.
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Bleeding tendency
Type I vWD patients have clearly impaired clotting but usually end up
leading a nearly normal life (trouble usually arises in the form of
dental surgery or troublesome menorrhagia/periods). Type III is the most
severe form of vWD and may have severe mucosal bleeding, no detectable
vWF antigen, and/or may have sufficiently low factor VIII that they have
occasional hemarthoses like mild hemophiliacs.
Classification
There are three described types of vWD - type I, type II and type III,
of which type I and II are inherited as autosomal dominant traits and
type III is inherited as autosomal recessive.
Although very rare, acquired forms of vWD have been described. A form of
vWD (type 2A) occurs in patients with aortic valve stenosis, leading to
gastrointestinal bleeding (Heyde's syndrome).
Treatment
Patients with vWD normally require no regular treatment. However, they
are always at increased risk for bleeding. Prophylactic treatment is
sometimes given for patients with vWD who are scheduled for surgery.
They can be treated with cyroprecipitate or with Factor VIII. Mild cases
of vWD can be trialled on 1-desamino-8-D-arginine vasopressin (DDAVP,
desmopressin), which works by raising the patient's own plasma levels of
vWF.
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