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Case Reports
. 2017 Mar;57(3):700-704.
doi: 10.1111/trf.13961. Epub 2016 Dec 26.

Severe cardiac iron toxicity in two adults with sickle cell disease

Hellen Oduor  1 , Caterina P Minniti  2 , Alessandra Brofferio  3 , Ahmed M Gharib  4 , Khaled Z Abd-Elmoniem  4 , Matthew M Hsieh  5 , John F Tisdale  5 , Courtney D Fitzhugh  1   5
Affiliations
Case Reports

Severe cardiac iron toxicity in two adults with sickle cell disease

Hellen Oduor et al. Transfusion. 2017 Mar.

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.

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Conflict of interest statement

Conflict of Interest: The authors have disclosed no conflicts of interest.

Figures

Figure 1
Figure 1
Autopsy findings in Patient 1 which revealed diffuse siderosis in the a) Thyroid, b) Adrenals, c) Pancreas d) Kidney, e) Heart, and f) Liver.
Figure 2
Figure 2
Left ventricular ejection fraction and serum ferritin following chelation with intravenous deferoxamine.
See this image and copyright information in PMC

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