dr V K Khanna
Consultant Pediatrician

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  • 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.

Posted On: 08/09/2018

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