Goal of chelation therapy is to decrease tissue iron to a safe level and to minimise level of non-transferrin bound iron (NTBI) which is mainly responsible for iron toxicity.
To minimise the levels of non-transferrin bound iron, a 24 hours chelation coverage is ideal
Chelation therapy should be started when:
serum ferritin is more than 1000 ng/ml
after 15-20 transfusions
Aim is to maintain serum ferritin < 1000 ng/ml.
Desferal (DFO) is a time tested and effective iron chelator. It is administered in the dose of 20-50 mg/ kg/day as subcutaneous infusion with the help of a pump. Desferal is less often used these days, because of high cost, need for parenteral administration and poor compliance.
Deferiprone (DFP) is cheap and orally effective and is a better iron chelator for cardiac iron overload than DFO . The recommended dose is 50-100 mg/kg/day in 3-4 divided doses . Regular CBC monitoring every 2-4 weeks is required as there is risk of agranulocytosis (< 1%)
Deferasirox (DFX) is a new synthetic oral iron chelator with a long half- life (8-16 hrs) and provides 24 hours protection from NTBI with once a day dose. The recommended dose is 20-40 mg/kg/day and should be taken empty stomach dispersed in water or juice. It is effective and relatively safe. Renal and hepatic functions should be monitored every month
Splenectomy is less often required these days because of better modern day management and should be avoided if possible.