Haemophilia A/B

Question 1

how is haemophilia A and B inherited?

Answer 1

x linked recessive

Question 2

what is the pathophysiology of haemophilia A?

Answer 2

Factor 8 deficiency

Question 3

Clinical features?

Answer 3

1. haemoarthroises - bleeding into the joints
2. haematomas
3. prolonged bleeding after surgery

Question 4

Mx

Answer 4

1. Factor replacement therapy - Factor V111 / IX

2. Tanexamic acid - given during acute bleeds

Question 5

what is the pathophysiology of haemophilia B?

Answer 5

Factor 9 deficiency

Question 6

what is haemophilia B also known as and how common is it?

Answer 6

1. also known as Christmas disease?

2. 5 x less common than Haem A

Question 7

Results of blood tests :

Answer 7

1. prolonged APTT
2. normal;
   - bleeding time
   - thrombin time
   - prothrombin time