Haematology 13 - VWD + Haemophilia

Question 1

von Willebrand disease

Answer 1

most common inherited cause of abnormal bleeding

mainly auto dominant

type 3 most severe

Question 2

presentation of vwd

Answer 2

bleeding gums easily
nose bleeds
heavy menstruals
surgical xs bleeds

FH

Question 3

managing VWD

Answer 3

day to day none

if bleeding

desmopressin
VWF infusion
factor 8 infusion

women - treat their heavy periods as would

Question 4

haemophilia A and B

Answer 4

A - def in factor VIII

B - def in factor IX

X linked recessive
men nearly always

Question 5

features of haemophilia

Answer 5

deep joint bleeding (haemarthrosis)
muscle bleeds
can occur elsewhere and have spontaneous bleeds without trauma

Question 6

managing haemophilias

Answer 6

infuse clotting factors

although can make Abs vs these

also tranexamic acid
desmopressin