Von Willebrand's Disease

Question 1

Definition

Answer 1

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

Question 2

Types

Answer 2

o Type 1 - the von Willebrand factor works well but there isn’t enough of it

o Type 2 - there are normal levels of von Willebrand factor but it is abnormal and
doesn’t function correctly

o Type 3 - there is NO von Willebrand factor

Question 3

Aetiology

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 its degradation

Question 4

Presenting symptoms/signs on physical examination

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

Investigations

Answer 5

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