Iron Chelation Therapy in Thalassemia

Introduction to Iron Chelators

Deferiprone is the preferred agent for patients with cardiac iron overload or heart failure, while deferasirox offers superior convenience for maintenance therapy in stable patients, and deferoxamine remains essential for acute cardiac decompensation requiring intensive chelation 1, 2

The American Heart Association recommends deferoxamine administration subcutaneously at 20-50 mg/kg/day via portable pump over 8-14 hours for 5-7 days per week, or continuous intravenous infusion when intensive chelation is needed 1, 3
Deferiprone is administered orally at 75-100 mg/kg/day divided into three daily doses, with a plasma half-life of 1.5-2.5 hours, requiring three-times-daily dosing 1, 4
Deferasirox is administered orally at 20-40 mg/kg/day once daily, given as dispersible tablets dissolved in water or juice at least 30 minutes before meals, with an elimination half-life of 8-16 hours 1

Deferiprone demonstrates superior cardiac iron clearance compared to other agents and is the drug of choice for patients with cardiac T2* <20 ms or established heart failure 2, 5
Cardiac iron removal is extremely slow even with intensive chelation, requiring several years of treatment with monitoring by cardiac T2* MRI 2, 3

For acute cardiac decompensation or heart failure, initiate deferiprone immediately at 75 mg/kg/day in three divided doses 2
Consider continuous intravenous deferoxamine at 50-60 mg/kg/day for rapid cardiac iron removal 2, 3
After stabilization, transition to combination therapy with daily subcutaneous deferoxamine plus daily oral deferiprone 2

Serum ferritin should be monitored every 3 months, targeting levels <1000 ng/mL 3, 5
Cardiac T2* MRI should be performed annually in transfusion-dependent patients starting at age 10 3
Liver iron concentration should be monitored annually by MRI 3
Annual electrocardiography and echocardiography are recommended 3

Delayed cardiac iron clearance can occur even with intensive chelation, and treatment must continue long-term with regular T2* monitoring to prevent premature discontinuation 2, 3, 5
Inappropriate agent selection, such as using deferasirox in acute heart failure or renal impairment, can worsen outcomes 2, 5
Failing to use deferiprone in cardiac iron overload misses the most effective cardiac-specific agent 2, 5
Not monitoring neutrophils weekly with deferiprone risks life-threatening agranulocytosis 1, 4