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PLoS One. 2017 Mar 3;12(3):e0172147. doi: 10.1371/journal.pone.0172147. eCollection 2017.

Cardiac iron overload in chronically transfused patients with thalassemia, sickle cell anemia, or myelodysplastic syndrome.

Author information

Pediatrics Department, Necker Children's Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France.
Laboratory of Excellence GR-Ex, Paris, France.
Biotherapy Department, Necker Children's Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France.
Biotherapy Clinical Investigation Center, Groupe Hospitalier Universitaire Ouest, Assistance Publique-Hôpitaux de Paris, INSERM, Paris, France.
Hematology Department, Hôpital d'Adultes du Brabois, Vandoeuvre les Nancy, France.
Centre Henri Becquerel, Rouen, France.
Pediatric Oncology and Hematology Unit, Hôpital Charles Nicolle, Rouen, France.
Department of Biochemistry, Hôpital Charles Nicolle, Rouen, France.
Service d'Hématologie Clinique et de Thérapie Cellulaire, CHU, Limoges, France.
Service des Maladies du Sang, Hôpital Haut-Levêque, Pessac, France.
Paris Descartes-Sorbonne Paris Cité University, Imagine Institute, Paris, France.
INSERM UMR 1163, Laboratory of Human Lymphopoiesis, Paris France.
Département de Pharmacologie clinique et toxicologique, CHU, Bordeaux, France.
Paris Descartes Clinical Research Unit, Necker Children's Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France.
INSERM UMR 1149/ERL. CNRS 8252, Centre de Recherche sur l'inflammation, Paris, France.
French center for Porphyria, Louis Mourier Hospital, Assistance Publique-Hôpitaux de Paris, Colombes, France.
Radiology Department, Hopital Huriez, CHRU, Lille, France.
Hématologie clinique, Hôpital Saint Vincent de Paul, Université Catholique de Lille, Lille, France.


The risk and clinical significance of cardiac iron overload due to chronic transfusion varies with the underlying disease. Cardiac iron overload shortens the life expectancy of patients with thalassemia, whereas its effect is unclear in those with myelodysplastic syndromes (MDS). In patients with sickle cell anemia (SCA), iron does not seem to deposit quickly in the heart. Our primary objective was to assess through a multicentric study the prevalence of cardiac iron overload, defined as a cardiovascular magnetic resonance T2*<20 ms, in patients with thalassemia, SCA, or MDS. Patient inclusion criteria were an accurate record of erythrocyte concentrates (ECs) received, a transfusion history >8 ECs in the past year, and age older than 6 years. We included from 9 centers 20 patients with thalassemia, 41 with SCA, and 25 with MDS in 2012-2014. Erythrocytapharesis did not consistently prevent iron overload in patients with SCA. Cardiac iron overload was found in 3 (15%) patients with thalassemia, none with SCA, and 4 (16%) with MDS. The liver iron content (LIC) ranged from 10.4 to 15.2 mg/g dry weight, with no significant differences across groups (P = 0.29). Abnormal T2* was not significantly associated with any of the measures of transfusion or chelation. Ferritin levels showed a strong association with LIC. Non-transferrin-bound iron was high in the thalassemia and MDS groups but low in the SCA group (P<0.001). Hepcidin was low in thalassemia, normal in SCA, and markedly elevated in MDS (P<0.001). Two mechanisms may explain that iron deposition largely spares the heart in SCA: the high level of erythropoiesis recycles the iron and the chronic inflammation retains iron within the macrophages. Thalassemia, in contrast, is characterized by inefficient erythropoiesis, unable to handle free iron. Iron accumulation varies widely in MDS syndromes due to the competing influences of abnormal erythropoiesis, excess iron supply, and inflammation.

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