Haemophilia

Question 1

What type of biochemical coagulopathy is associated with Haemophilia A?

Answer 1

Long APTT - intrinsic, normal PT

APTT refers to activated partial thromboplastin time, which is prolonged in Haemophilia A.

Question 2

What is the treatment for Haemophilia A?

Answer 2

Recombinant F8

Recombinant Factor VIII (F8) is used to replace the missing clotting factor in Haemophilia A patients.

Question 3

What medication can be used alongside Recombinant F8 for haemophilia treatment?

Answer 3

Desmopressin

Desmopressin is often used to help release stored Factor VIII from the endothelial cells.

Question 4

What is the dosage of Recombinant F8 for minor to moderate bleeding?

Answer 4

30-50u/kg

This dosage is recommended for minor to moderate bleeding episodes.

Question 5

What is the recommended dosage of Recombinant F8 for major bleeding or CNS bleeding?

Answer 5

50-75u/kg

Higher dosages are crucial for severe bleeding situations.

Question 6

In which type of bleeding should you consider using Desmopressin?

Answer 6

Mild bleeding

Desmopressin is particularly useful in cases of mild bleeding.

Question 7

What is the intravenous/subcutaneous dosage of Desmopressin?

Answer 7

0.3mcg/kg

This dosage is administered for effective treatment.

Question 8

What is the intravenous dosage of TXA?

Answer 8

15mg/kg

TXA is especially effective for managing epistaxis and mucosal bleeds.

Question 9

Should TXA be used in cases of haematuria?

Answer 9

No

TXA is contraindicated in patients with haematuria.

Question 10

What factor if deficient in Hemophilia B?

Answer 10

Factor IX