Iron Chelation Therapy in Sickle Cell Anemia

Initiation Criteria

• The American Society of Hematology recommends initiating iron chelation therapy when serum ferritin reaches ≥1,000 ng/mL in patients with sickle cell disease receiving chronic transfusions 1
• Initiate chelation when transfusion burden is ≥2 units per month sustained for more than one year, regardless of ferritin level, as cardiac abnormalities develop after >100 units and liver iron accumulation occurs after >24 units 1

Special Clinical Scenarios

• The American College of Cardiology recommends initiating chelation in stem cell transplant candidates even with moderate iron overload, as ferritin >1,000 ng/mL at transplant is associated with higher mortality and increased hepatic complications 1, 2
• Initiate chelation when there is evidence of organ dysfunction from iron overload, regardless of ferritin level or life expectancy 1, 2

Monitoring Requirements

• The National Institutes of Health recommends performing MRI for liver iron content every 1-2 years using validated R2, T2, or R2 methods 3
• Consider cardiac T2* MRI screening for patients with high iron burden (LIC >15 mg/g for ≥2 years), evidence of end-organ damage, or cardiac dysfunction 3