Haematology

Question 1

What are important points on history for a patient with haemophilia and bleeding?

Answer 1

• Type and severity of haemophilia
• What caused the bleeding and what was injured
• Do they have a home management plan and have they already administered factor replacement
• Are they on prophylactic factor replacement, how much and when was it last given
• Do they have a known inhibitor (ie need for bypassing agent)
• What is their weight (for dosing factor replacement)
• Does the patient have a portacath/PICC line already for access

Question 2

What is the difference between Haemophilia A and B

Answer 2

A = Factor 8 (VIII), usually X linked
B = Factor 9 (IX)

Question 3

What analgesics should not be given to patients with haemophilia?

Answer 3

NSAID’s and Aspirin due to bleeding risk

Question 4

Other than factor replacement what else can be given to help with bleeding in haemophilia patients?

Answer 4

• Tranexamic acid 25mg/kg
• Desmopressin/DDAVP in mild haemophilia A (VIII and VwF release)
• Bypass agents such as recombinant factor 7 (Novoseven)

Question 5

What is the managment of mild bleeding in Haemophilia A? Major bleeding?

Answer 5

Mild
- eg haemarthrosis
- Aiming to get to 50% VIII activity
- 1unit/kg = 2% increase in activity
- Thus 30 units/kg should increase VIII activity to at least 60%
- Often given 40units/kg at RCH for minor bleeding/joints
- Desmopressin 0.3mcg/kg SC
- TXA 25mg/kg PO for mucosal and epistaxis bleeding

Severe
- eg intracranial bleed
- Aiming to get to 100% VIII activity
- at least 50units/kg, RCH states 75units/kg

Dosage of IX factor replacement in Haemophilia B is roughly double the dose of Haemophilia A
- Mild 30 HA, 60Hb
- Major 50 HA, 100HB
- Life threatening 75HA, 125HB

Question 6

What is the severity grading for haemophilia A VIII levels?

Answer 6

Mild
- 5-40% VIII activity
- Significant bleeding only occurs with major trauma or surgery/procedures

Moderate
- 1-5%
- Bleeding after minor injury
- Spontaneous haemarthrosis
- Severe bleeds with moderate trauma or surgery/procedures

Severe
- <1%
- Can have spontaneous severe bleeds or after minor injury
- Often have spontaneous haemarthrosis and muscle bleeds

Question 7

What is the general management of Haemophilia associated muscle/joint bleeds?

Answer 7

PRICE
P- Protection (immobilise in position of comfort)
+ RICE

Paracetamol
Nitrous oxide as an adjunct if cannulation required

Don’t use NSAID’s/Aspirin