Von willebrand disease

Question 1

What is it?

What does von willebrand factor do?

Answer 1

Von Willebrand’s disease is the most common inherited bleeding disorder

• promotes platelet adhesion to damaged endothelium
• carrier molecule for factor VIII

Question 2

Clinical features

Answer 2

Bleeding gums with brushing
Nose bleeds (epistaxis)
Heavy menstrual bleeding (menorrhagia)
Heavy bleeding during surgical operations
Family history of heavy bleeding or von Willebrand disease is very relevant.

Question 3

Types

Answer 3

type 1: partial reduction in quantity of vWF (80% of patients)
type 2*: abnormal form of vWF
type 3**: total lack of vWF (autosomal recessive)

Question 4

Investigation

Answer 4

prolonged bleeding time

APTT may be prolonged

factor VIII levels may be moderately reduced

defective platelet aggregation with ristocetin

Question 5

Management

Answer 5

Tranexamic acid for mild bleeding

Desmopressin (DDAVP): raises levels of vWF by inducing release of vWF from Weibel-Palade bodies in endothelial cells

Factor VIII concentrate