Von Willebrand disease

Question 1

How is VW disease inherited?

Answer 1

1. autosomal dominant with incomplete penetration

Question 2

What is the pathophysiology of VW disease?

Answer 2

1. deficiency in von willebrand factor

2. Von willebrand factor helps coagulation by promoting platelet interaction with endothelium

Question 3

Clinical features of Von willebrand disease?

Answer 3

1. nose bleeds / GI bleeds
2. menorrhagia
3. bleeding post trauma/surgery

Question 4

Lab results - blood tests

Answer 4

1. Prolonged :
   - platelet aggregation
   - bleeding time
2. Normal PT time
3. Prolonged/normal APTT time

Question 5

Mx of Von willebrand disease?

Answer 5

1. tranexamic acid for mild bleeding

2. desmopressin - raises levels of vWf