Haem - Von Willebrand Disease Flashcards
What is VWD?
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
What happens in VWD?
Deficiency, absence or malfunctioning of a VWF (glycoprotein)
Important in platelet adhesion and aggregation in damaged vessels
What are the 3 types of VWD?
Type 1
Partial deficiency of VWF
Most common and mildest
Type 2
Reduced function of VWF
Type 3
Complete deficiency
Most rare and severe
How does VWD present?
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
How is VWD diagnosed?
Based on history of abnormal bleeding
Bleeding assessment tools
Investigations
How is VWD managed?
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
What does desmopressin do in management of VWD?
Stimulates release of VWF from endothelial cells
What can be given for menorrhagia?
Tranexamic acid
Mefenamic acid
Mirena coil
COCP
Norethisterone
Hysterectomy - last line