Von Willebrand's disease

Question 1

how is Von Willebrand’s disease inherited?

Answer 1

autosomal dominant

Question 2

What type of bleeds are common in Von Willebrands?

Answer 2

epistaxis and menorrhagia

=> haemoarthroses and muscle haematomas are rare

Question 3

What is the usual role of vWF

Answer 3

glycoprotein which forms massive multimers

promotes platelet adhesion to damaged endothelium

carrier molecule for factor VIII

Question 4

What are the 3 types of Von Willebrands disease?

Answer 4

type 1: partial reduction in vWF
type 2: abnormal form of vWF
type 3: total lack of vWF (autosomal recessive)

Question 5

Investigations used to diagnose Von willebrand’s disease

Answer 5

Coagulation:
- APTT may be prolonged

factor VIII levels may be moderately reduced

Question 6

Management of von willebrand’s disease

Answer 6

• tranexamic acid for mild bleeding
• desmopressin raises levels of vWF by inducing release of vWF from Weibel-Palade bodies in endothelial cells
• factor VIII concentrate