Bleeding - Inherited

Question 1

What do defects in platelet-vessel wall interaction involve?

Answer 1

Weakened ability of platelet to bind through site of injury (either through collagen or vWf). Can be defect in protein, in vessel wall or on platelet surface

Question 2

What do defects in platelet-platelet interaction involve?

Answer 2

1. Platelets will bind to site of injury but platelets don’t stick together
2. No aggregation
3. No haemostatic plug

Question 3

What is Von Willebrand syndrome an example of?

Answer 3

Defects in platelet-vessel wall interaction

Question 4

What is Von Willebrand disease?

Answer 4

Deficiency / defective vWf. Most common inherited bleeding disorder

Question 5

What is vWf required for?

Answer 5

Platelet adhesion and activation

Question 6

What factor is vWf bound to in circulation? How does a deficiency in vWf affect this factor?

Answer 6

F8. Deficiency reduces carrying capacity for F8

Question 7

What drug can be used to treat vWf? How does it work?

Answer 7

Desmopressin

Releases vWf from endothelium or platelets