Von Willebrand Factor Deficiecny Flashcards
what is the most common inherited cause of bleeding?
Von Williebrand Factor deficiency
what is the inheritence- Von Williebrand Factor deficiency
autosomal dominant
what does Von Williebrand Factor deficiency affect?
it causes a deficiecny, absence or malfucntion of the glycoprotein Von Williebrand Factor which allows platelet adhesion AND binds to clotting factor VIII
types of Von Williebrand Factor deficiency?
type 1-3
3 being worst
presentation VWFD?
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
investigations for VWFD?
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)
mangement of VWFD?
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