Von Willebrand Disease

Question 1

What is VWD?

Answer 1

Most common inherited cause of abnormal bleeding and prolonged bleeding

Many genetic causes, most are autosomal dominant

Rare version secondary to underlying disease e.g. leukaemia

Question 2

How is VWD inherited?

Answer 2

Autosomal dominant

Question 3

What happens in VWD?

Answer 3

Deficiency, absence or malfunctioning of a VWF (glycoprotein)

VWF transports Factor VIII and it increases its half-life

Factor IIa releases Factor VIII from VWF

Without VWF there is a reduction in clotting leading to increased bleeding

Question 4

What are the 3 types of VWD?

Answer 4

Type 1
Partial deficiency of VWF
Most common and mildest

Type 2
Reduced function of VWF

Type 3
Complete deficiency
Most rare and severe

Question 5

How does VWD present?

Answer 5

History of easy, prolonged or heavy bleeding
- Bleeding gums with brushing
- Epistaxis
- Easy bruising
- Menorrhagia
- Heavy bleeding during and after operations
- Family history of heavy bleeding

Question 6

How is VWD diagnosed?

Answer 6

Based on history of abnormal bleeding

Bleeding assessment tools

Investigations

Question 7

How is VWD managed?

Answer 7

Does not need daily treatment

Managed in response to significant bleeding or trauma or to prepare for operations

• Desmopressin
• Tranexamic acid
• VW factor infusion
• Factor VIII + VWF infusion

Question 8

What does desmopressin do in management of VWD?

Answer 8

Stimulates release of VWF from endothelial cells

Question 9

What can be given for menorrhagia?

Answer 9

Tranexamic acid
Mefenamic acid
Mirena coil
COCP
Norethisterone

Hysterectomy - last line

Question 10

What do investigations show in VWD?

Answer 10

Isolated rise in APTT

No effect on PT or bleeding time