Iron Chelation

Question 1

What pre-transfusion Hb should you aim for, when treating thalassemia?

Answer 1

95-100mcg/L

Question 2

How often should you transfuse a thalassemia patient?

Answer 2

2-4 times per week

Question 3

When is iron chelation therapy indicated?

Answer 3

When serum ferritin >1000mcg/L

Question 4

Describe the route of administration, the dosage and side effects of Deferasirox.

Answer 4

Oral, 20-40mg/kg (1x day), rash, hepaptitis, renal impairment

Question 5

Describe the route of administration, the dosage and side effects of Desferorroxamine.

Answer 5

SC infusion, 20-50mg/kg/day (8-12 hours, 5-7 days per week), vertebral dysplasia, retinopathy, infection.

Question 6

Describe the route of administration, the dosage and side effects of Deferiprone.

Answer 6

Oral, 5-100 mcg/kg/day (3x daily), agranulocytosis, hepatic impairment.

Question 7

Name 2 major advantages and 2 major disadvantages of Desferroxamine.

Answer 7

Advantages: prevents and reverses heart failure, 3 decades experience. Disadvantages: parenteral administration limits compliance, dose-dependent toxicity (ocular, auditory, skeletal)

Question 8

Name 2 major advantages and 2 major disadvantages of Deferiprone.

Answer 8

Advantages: cardiac protection, oral administration. Disadvantages: 3x daily, unpredictable control of body iron, ( can cause agranulocytosis and zinc deficiency)

Question 9

Name 2 major advantages and 2 major disadvantages of Deferasirox.

Answer 9

Advantages: oral administration once daily, control of body iron. Disadavantges- long term data lacking on cardiac protection and other toxicities.

Question 10

Name 3 ways to monitor iron overload

Answer 10

Serum ferritin (>2500 mcg/L= problem) with APP, liver biopsy (rare), Ferriscan/cardiac/hepatic MRI.