vonWillebrand's Disease

Question 1

What does vWF do?

Answer 1

1. Brings platelets into contact with exposed subendothelium
2. Make the platelets bind to eachother
3. Bind to factor VIII, protecting it from destruction in the circulation

Question 2

How are most cases inherited?

Answer 2

Autosomal dominant

Question 3

How does it present?

Answer 3

• Epistaxis
• Menorrhagia
• Bruising
• Bleeding gums
  Like a platelet disorder

Question 4

What are the types of vWD

Answer 4

Type 1 - Partial reduction (most common)
Type 2 - Abnormal form of vWF
Type 3 - Total lack of vWF (autosomal recessive)

Question 5

What investigations are required?

Answer 5

• Prolonged bleeding time
• Raised APTT
• Low factor VIIIc
• Low vWF antigen
• Normal INR and normal platelets

Question 6

What is the management?

Answer 6

• Tranexamic acid for mild bleeds
• Desmopressin raises levels of vWF
• Factor VIII concentrate