Von Willebrand Factor Deficiecny

Question 1

what is the most common inherited cause of bleeding?

Answer 1

Von Williebrand Factor deficiency

Question 2

what is the inheritence- Von Williebrand Factor deficiency

Answer 2

autosomal dominant

Question 3

what does Von Williebrand Factor deficiency affect?

Answer 3

it causes a deficiecny, absence or malfucntion of the glycoprotein Von Williebrand Factor which allows platelet adhesion AND binds to clotting factor VIII

Question 4

types of Von Williebrand Factor deficiency?

Answer 4

type 1-3

3 being worst

Question 5

presentation VWFD?

Answer 5

heavy bleeding + bruising

history of unusually easy, prolonged or heavy bleeding :
-bleeding gums with bruising
-nose bleeds (epistaxis)
-heavy menstrual bleeding (menorrhagia)
-heavy bleeding during surgical operations

Question 6

investigations for VWFD?

Answer 6

Look for FH of VWFD (it has autosomal dominant inheritance) or prolonged bleeding

Platelets= normal
PT= normal
APTT= prolonged (due to interaction with clotting factor VIII)

Question 7

mangement of VWFD?

Answer 7

Doesn’t require day to day treatment – just when required (major trauma, pre-op):

-desmopressin (used to stimulate the release of VWF)
-VWF can also be infused
-Factor 8 is often infused along with plasma-derived VWF

If heavy periods – tranexamic acid, mefenamic acid, norethisterone, COC, mirena coil, hysterectomy