title: Longitudinal MRI and Ferritin Monitoring of Iron Overload in Chronically Transfused and Chelated Children With Sickle Cell Anemia and Thalassemia Major.
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.
authors.affiliation: *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.
authors.forename: Mélodie
authors.initials: M
authors.lastname: Aubart
authors.forename: Phalla
authors.initials: P
authors.lastname: Ou
authors.forename: Caroline
authors.initials: C
authors.lastname: Elie
authors.forename: Carla
authors.initials: C
authors.lastname: Canniffe
authors.forename: Shelby
authors.initials: S
authors.lastname: Kutty
authors.forename: Vincent
authors.initials: V
authors.lastname: Delos
authors.forename: Christine
authors.initials: C
authors.lastname: Graffigne
authors.forename: Mariane
authors.initials: M
authors.lastname: de Montalembert
authors.forename: Valentine
authors.initials: V
authors.lastname: Brousse
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000097:blood / Q000628:therapy*; D001803:Blood Transfusion; D015913:Chelation Therapy; D002648:Child; D002675:Child, Preschool; D005260:Female; D005293:Ferritins / Q000097:blood*; D006321:Heart / Q000000981:diagnostic imaging; D006801:Humans; D019190:Iron Overload / Q000097:blood / Q000000981:diagnostic imaging*; D008099:Liver / Q000000981:diagnostic imaging; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D017086:beta-Thalassemia / Q000097:blood / Q000628:therapy*
chemical_list: D005293:Ferritins