Haematology

Question 1

What is haemophilia? Different types?

Answer 1

Haemophilia is an X linked recessive condition in which there are deficiencies in clotting factors resulting in excessive bleeding responses to even minor traumas.

There is Haemophilia A and Haemophilia B

Question 2

What clotting factor is deficient in Haemophilia A?

Answer 2

Factor VIII

Question 3

What clotting factor is deficient in Haemophilia B?

Answer 3

Factor IX

Question 4

Presentation of Haemophilias?

Answer 4

Most cases present in neonates/early childhood with spontaneous bleeding into joints and muscles, cord bleeding excessively, intracranial haemorrhage or haematomas.

Question 5

How do you manage Haemophilia A and Haemophilia B?

Answer 5

Replacing deficient clotting factors
Factor VIII for Haemophilia A
Factor IX for Haemophilia B

Antifibrinolytics (tranexamic acid) to stop immediate bleeding

Can also give desmopressin to stimulate von Willebrand factor *not in Acute bleeds