Von Willebrand's Disease

Question 1

Define Von Willebrand Disease?

Answer 1

Bleeding disorder which may present with mucocutaneous bleeding (mouth, epistaxis, menorrhagia), increased bleeding after trauma and easy bruising

Question 2

What are the three types of von Willebrand Disease?

Answer 2

Type 1 - the von Willebrand factor works well but there isn’t enough of it
Type 2 - there are normal levels of von Willebrand factor but it is abnormal and doesn’t function correctly
Type 3 - there is NO von Willebrand factor

Question 3

What is the aetiology of von Willebrand Disease?

Answer 3

Caused by abnormality in the expression/function of vWF
Usually autosomal dominant
vWF is an adhesive bridge between platelets and the damaged subendothelial collagen
vWF also binds to factor VIII and prevents it degradation

Question 4

What are the signs and presenting symptoms of von Willebrand Disease?

Answer 4

Easy bruising
Epistaxis - hard to stop
Prolonged bleeding from gums after dental procedures
Heavy or prolonged menstrual bleeding
Blood in stools
Blood in urine
Heavy bleeding from a cut or other accident

Question 5

What investigations would you do for von Willebrand Disease?

Answer 5

Bleeding time - HIGH
APTT - HIGH 
Factor VIII - LOW 
vWF - LOW 
Ristocetin cofactor - reduced platelet aggregation by vWF in the presence of ristocetin