Von Willebrand Disease

Question 1

What is the most common inherited cause of abnormal and prolonged bleeding?

Answer 1

Von Willebrand disease

Question 2

What is the pathophysiology of von willebrand disease?

Answer 2

Deficiency, absence of malfunctioning of glycoprotein von willebrand factor

Question 3

What are the types of von willebrand disease?

Answer 3

Type 1 - partial deficiency of VWF
Type 2 - reduced function of VWF
Type 3 - complete deficiency of VWF

Question 4

What is the most common type of von willebrand disease?

Answer 4

Type 1

Question 5

What is the presentation of von willebrand disease?

Answer 5

Bleeding gums
Nosebleeds
Easy bruising
Menorrhagia
Heavy bleeding during and aftery surgery

Question 6

What is the management of von willebrand disease?

Answer 6

Desmopression
Tranexamic acid
VWF infusion
Factor VIII plus VWF infusion

Question 7

How does desmopressin work in von willebrand disease?

Answer 7

Stimulates the release of VWF from endothelial cells