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Updated: Mar 16, 2026
Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
Published on: March 14, 2017
Longitudinal MRI and Ferritin Monitoring of Iron Overload in Chronically Transfused and Chelated Children With Sickle
Mélodie Aubart1, Phalla Ou, Caroline Elie
1*Department of Pediatrics ∥Clinical Research Unit †Department of Radiology, Hôpital Universitaire Necker Enfants Malades, APHP ‡Paris Descartes University §UMR 8145, MAP5, Paris, France ¶Division of Pediatric Cardiology, University of Nebraska Medical Center and Children's Hospital and Medical Center, Omaha, NE.
Insights
Iron overload remains a risk for children receiving regular blood transfusions, even with chelation therapy. While ferritin levels correlate with liver iron, magnetic resonance imaging (MRI) is crucial for accurate monitoring.
Area of Science:
- Pediatric Hematology
- Medical Imaging
- Pharmacology
Background:
- Chronic blood transfusions in children with sickle cell anemia (SCA) and thalassemia major (TM) can lead to iron overload.
- Chelation therapy is used to manage iron burden, but its effectiveness requires careful monitoring.
- Accurate assessment of iron levels is vital to prevent organ damage in these pediatric populations.
Purpose of the Study:
- To longitudinally assess the relationship between serum ferritin levels and hepatic/cardiac iron levels measured by T2* magnetic resonance imaging (MRI).
- To evaluate the predictive value of ferritin trends for iron overload in children with SCA and TM undergoing chelation therapy.
- To compare iron burden and monitoring accuracy between SCA and TM patients receiving deferasirox.
Main Methods:
- Longitudinal study of 30 SCA and 7 TM children receiving deferasirox chelation.
- Regular monitoring of serum ferritin levels.
- Annual hepatic T2* MRI and periodic cardiac T2* MRI to quantify iron content.
Main Results:
- A significant correlation was observed between serum ferritin levels and transfusion iron intake, despite chelation.
- Serum ferritin strongly correlated with liver iron content (assessed by hepatic T2* MRI) in both SCA and TM patients.
- Cardiac iron overload was absent in SCA patients but present in a few TM patients; ferritin trends did not reliably predict cardiac iron.
Conclusions:
- Iron overload remains a significant concern in chronically transfused children, even with chelation therapy.
- Serum ferritin levels are a useful indicator of liver iron burden in chelated children with SCA and TM.
- T2* MRI is the preferred method for monitoring iron burden, especially for cardiac iron, when feasible.
Abstract:
Iron overload is an ineluctable complication in chronically transfused children warranting accurate assessment to avoid related morbidity. We investigated longitudinally the relationships between ferritin levels and hepatic and cardiac T2* magnetic resonance imaging (MRI) in a cohort of chronically transfused children receiving chelation therapy. Thirty children with sickle cell anemia (SCA) and 7 with thalassemia major (TM) chelated similarly by deferasirox were analyzed. Sex ratio, age, median duration of transfusion programs (5 y; range, 2 to 14 y), median transfusion iron intake 0.54 mg/kg/d (range, 0.27 to 0.74 mg/kg/d), and median ferritin level (1550 mg/L; range, 184 to 6204 mg/L) were comparable in TM and SCA. A significant relation was found between ferritin level and transfusion iron intake (P<0.001) despite chelation therapy. Analysis of 73 hepatic T2* MRI performed yearly demonstrated severe hepatic iron overload (≥14 mg/g) in 38.3% cases and a strong relationship between serum ferritin level and liver iron content both in SCA and TM (P<0.001). Analysis of 55 cardiac T2* MRI measurements found no cardiac overload in patients with SCA. Cardiac iron overload was moderate in 4 cases and severe in 1 case of TM. In almost half the cases, ferritin trend correctly predicted hepatic iron trend, both in patients with SCA and TM but failed to predict cardiac iron trend, notably in TM patients. Despite chelation therapy, iron burden in chronically transfused patients remains a threat. Ferritin levels are associated with liver iron overload in chelated children with SCA and TM, but iron burden should be best monitored with MRI whenever the setting allows it.
