title: Severe cardiac iron toxicity in two adults with sickle cell disease.
abstract: Use of chronic blood transfusions as a treatment modality in patients with blood disorders places them at risk for iron overload. Since patients with β-thalassemia major (TM) are transfusion-dependent, most studies on iron overload and chelation have been conducted in this population. While available data suggest that compared to TM, patients with sickle cell disease (SCD) have a lower risk of extrahepatic iron overload, significant iron overload can develop. Further, previous studies have demonstrated a direct relationship between iron overload and morbidity and mortality rates in SCD. However, reports describing the outcome for patients with SCD and cardiac iron overload are rare.
  
  
  
  We performed a retrospective analysis and identified two SCD patients with cardiac iron overload. We provide detailed descriptions of both cases and their outcomes.
  
  
  
  Serum ferritin levels ranged between 17,000 and 19,000 μg/L. Both had liver iron concentrations in excess of 35 mg of iron per gram of dried tissue as well as evidence of cardiac iron deposition on magnetic resonance imaging. One patient died of an arrhythmia and had evidence of severe multiorgan iron overload via autopsy. On the other hand, after appropriate therapy, a second patient had improvement in cardiac function.
  
  
  
  Improper treatment of iron overload in SCD can lead to a fatal outcome. Alternatively, iron overload may potentially be prevented or reversed with judicious use of blood transfusions and early use of chelation therapy, respectively.
authors.affiliation: Hematology Branch, National Heart, Lung, and Blood Institute, Bethesda, Maryland.
authors.forename: Hellen
authors.initials: H
authors.lastname: Oduor
authors.affiliation: Division of Hematology, Department of Medicine, Einstein College of Medicine, Bronx, New York.
authors.forename: Caterina P
authors.initials: CP
authors.lastname: Minniti
authors.affiliation: Cardiovascular Branch, National Heart, Lung, and Blood Institute, Bethesda, Maryland.
authors.forename: Alessandra
authors.initials: A
authors.lastname: Brofferio
authors.affiliation: Biomedical and Metabolic Imaging Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland.
authors.forename: Ahmed M
authors.initials: AM
authors.lastname: Gharib
authors.affiliation: Biomedical and Metabolic Imaging Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland.
authors.forename: Khaled Z
authors.initials: KZ
authors.lastname: Abd-Elmoniem
authors.affiliation: Molecular and Clinical Hematology Branch, National Heart, Lung, and Blood Institute, Bethesda, Maryland.
authors.forename: Matthew M
authors.initials: MM
authors.lastname: Hsieh
authors.affiliation: Molecular and Clinical Hematology Branch, National Heart, Lung, and Blood Institute, Bethesda, Maryland.
authors.forename: John F
authors.initials: JF
authors.lastname: Tisdale
authors.affiliation: Hematology Branch, National Heart, Lung, and Blood Institute, Bethesda, Maryland.|Molecular and Clinical Hematology Branch, National Heart, Lung, and Blood Institute, Bethesda, Maryland.
authors.forename: Courtney D
authors.initials: CD
authors.lastname: Fitzhugh
mesh_terms: D000328:Adult; D000755:Anemia, Sickle Cell / Q000097:blood / Q000150:complications / Q000503:physiopathology / Q000628:therapy; D001145:Arrhythmias, Cardiac / Q000097:blood / Q000209:etiology / Q000503:physiopathology / Q000628:therapy; D005260:Female; D005293:Ferritins / Q000097:blood*; D006801:Humans; D007501:Iron / Q000097:blood*; D019190:Iron Overload / Q000097:blood / Q000209:etiology / Q000503:physiopathology / Q000628:therapy; D008297:Male
chemical_list: D005293:Ferritins; D007501:Iron