Severe cardiac iron toxicity in two adults with sickle cell disease

Hellen Oduor, Caterina P. Minniti, Alessandra Brofferio, Ahmed M. Gharib, Khaled Z. Abd-Elmoniem, Matthew M. Hsieh, John F. Tisdale, Courtney D. Fitzhugh

Research output: Contribution to journalArticle

6 Citations (Scopus)

Abstract

BACKGROUND: 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. STUDY DESIGN AND METHODS: We performed a retrospective analysis and identified two SCD patients with cardiac iron overload. We provide detailed descriptions of both cases and their outcomes. RESULTS: 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. CONCLUSION: 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.

Original languageEnglish (US)
JournalTransfusion
DOIs
StateAccepted/In press - 2016

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Iron Overload
Sickle Cell Anemia
Iron
beta-Thalassemia
Blood Transfusion
Cardiotoxicity
Chelation Therapy
Fatal Outcome
Ferritins
Cardiac Arrhythmias
Autopsy
Therapeutics
Magnetic Resonance Imaging
Morbidity

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology
  • Hematology

Cite this

Oduor, H., Minniti, C. P., Brofferio, A., Gharib, A. M., Abd-Elmoniem, K. Z., Hsieh, M. M., ... Fitzhugh, C. D. (Accepted/In press). Severe cardiac iron toxicity in two adults with sickle cell disease. Transfusion. https://doi.org/10.1111/trf.13961

Severe cardiac iron toxicity in two adults with sickle cell disease. / Oduor, Hellen; Minniti, Caterina P.; Brofferio, Alessandra; Gharib, Ahmed M.; Abd-Elmoniem, Khaled Z.; Hsieh, Matthew M.; Tisdale, John F.; Fitzhugh, Courtney D.

In: Transfusion, 2016.

Research output: Contribution to journalArticle

Oduor, H, Minniti, CP, Brofferio, A, Gharib, AM, Abd-Elmoniem, KZ, Hsieh, MM, Tisdale, JF & Fitzhugh, CD 2016, 'Severe cardiac iron toxicity in two adults with sickle cell disease', Transfusion. https://doi.org/10.1111/trf.13961
Oduor, Hellen ; Minniti, Caterina P. ; Brofferio, Alessandra ; Gharib, Ahmed M. ; Abd-Elmoniem, Khaled Z. ; Hsieh, Matthew M. ; Tisdale, John F. ; Fitzhugh, Courtney D. / Severe cardiac iron toxicity in two adults with sickle cell disease. In: Transfusion. 2016.
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