Long case guidelines

Question 1

Factor deficiency for haemophilia A

Answer 1

Factor 8

Question 2

Factor deficiency for haemophilia B

Answer 2

Factor 9

Question 3

Severity rating for haemophilia by factor amount

Answer 3

Severe <1%
Moderate 1-5%
Mild >5%

Question 4

Inheritance of haemophilia

Answer 4

X-linked recessive

Question 5

De novo rate of haemophilia

Answer 5

1/3

Question 6

Where does the bleeding occur most commonly in haemophilia?

Answer 6

Joints and muscles
Knees >50% of all events
Intramuscular 30%

Question 7

What intramuscular bleeding in haemophilia requires urgent intervention?

Answer 7

Psoas bleed

Question 8

Mechanism of action of desmopressin in haemophilia

Answer 8

Stimulates transient x increase in F VIII and iWF levels from storage sites (so can only be used in minor haemophilia)

Question 9

Side effects of DDAVP (desmopressin)

Answer 9

Hyponatraemia (must fluid restrict)
Fluid reention/weight gain
Tachyphylaxis
AMI/CVA in elderly patients

Question 10

Mechanism of action of antifibrinolytic agents

Answer 10

Tranexamic acid - stabilises the clot by inhibiting lysis by plasmin (highly high fibrinolytic activity in mucous membrane areas

Question 11

What kind of bleeding is tranexamic acid contraindicated for

Answer 11

Haematuria due to high risk of intrarenal clots