Von Willebrand's Disease

Question 1

What is the most common inherited bleeding disorder?

Answer 1

Von Willebrands

Question 2

In what genetic fashion is it inherited?

Answer 2

Autosomal dominant.

Question 3

Name the 3 different types of Von Willbrand Disease.

Answer 3

1. Type 1: Partial reduction in vWF (80% of patients) (AD)
2. Type 2: Abnormal form of vWF (AD)
3. Type 3: Total lack of vWF (autosomal recessive)

Question 4

What would you see on blood tests in someone with vWF?

Answer 4

• prolonged bleeding time.
• APTT may be prolonged.
• Factor VIII levels may be moderately reduced.

Question 5

How do you manage vWF?

Answer 5

1. Tranexamic acid for mild bleeding.
2. Desmopressin (DDAVP): raises levels of vWF by inducing release of vWF from endothelial cells.
3. Factor VIII concentrate.

Question 6

vWF is a carrier protein for what factor?

Answer 6

Factor 8.

Question 7

Answer 7

Question 8

Question 9

A 34-year-old man who is known to have type 1 von Willebrand’s disease asks for advice. He is due to have a tooth extracted at the dentist next week. Which one of the following is the most appropriate management to reduce the risk of bleeding?

• Mefanamic acid
• Vitamin K
• Desmopressin
• Factor VIII concentrate
• Factor VII concentrate

Answer 9

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