Cardiac iron overload in chronically transfused patients with thalassemia, sickle cell anemia, or myelodysplastic syndrome.
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 si...
| Main Authors: | , , , , , , , , , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
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Public Library of Science (PLoS)
2017-01-01
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| Series: | PLoS ONE |
| Online Access: | http://europepmc.org/articles/PMC5336214?pdf=render |
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| author | Mariane de Montalembert Jean-Antoine Ribeil Valentine Brousse Agnes Guerci-Bresler Aspasia Stamatoullas Jean-Pierre Vannier Cécile Dumesnil Agnès Lahary Mohamed Touati Krimo Bouabdallah Marina Cavazzana Emmanuelle Chauzit Amandine Baptiste Thibaud Lefebvre Hervé Puy Caroline Elie Zoubida Karim Olivier Ernst Christian Rose |
| author_facet | Mariane de Montalembert Jean-Antoine Ribeil Valentine Brousse Agnes Guerci-Bresler Aspasia Stamatoullas Jean-Pierre Vannier Cécile Dumesnil Agnès Lahary Mohamed Touati Krimo Bouabdallah Marina Cavazzana Emmanuelle Chauzit Amandine Baptiste Thibaud Lefebvre Hervé Puy Caroline Elie Zoubida Karim Olivier Ernst Christian Rose |
| author_sort | Mariane de Montalembert |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-04-11T22:46:46Z |
| format | Article |
| id | doaj.art-002d532590b14061a3fc1d1939ec6a2d |
| institution | Directory Open Access Journal |
| issn | 1932-6203 |
| language | English |
| last_indexed | 2024-04-11T22:46:46Z |
| publishDate | 2017-01-01 |
| publisher | Public Library of Science (PLoS) |
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| series | PLoS ONE |
| spelling | doaj.art-002d532590b14061a3fc1d1939ec6a2d2022-12-22T03:58:44ZengPublic Library of Science (PLoS)PLoS ONE1932-62032017-01-01123e017214710.1371/journal.pone.0172147Cardiac iron overload in chronically transfused patients with thalassemia, sickle cell anemia, or myelodysplastic syndrome.Mariane de MontalembertJean-Antoine RibeilValentine BrousseAgnes Guerci-BreslerAspasia StamatoullasJean-Pierre VannierCécile DumesnilAgnès LaharyMohamed TouatiKrimo BouabdallahMarina CavazzanaEmmanuelle ChauzitAmandine BaptisteThibaud LefebvreHervé PuyCaroline ElieZoubida KarimOlivier ErnstChristian RoseThe 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.http://europepmc.org/articles/PMC5336214?pdf=render |
| spellingShingle | Mariane de Montalembert Jean-Antoine Ribeil Valentine Brousse Agnes Guerci-Bresler Aspasia Stamatoullas Jean-Pierre Vannier Cécile Dumesnil Agnès Lahary Mohamed Touati Krimo Bouabdallah Marina Cavazzana Emmanuelle Chauzit Amandine Baptiste Thibaud Lefebvre Hervé Puy Caroline Elie Zoubida Karim Olivier Ernst Christian Rose Cardiac iron overload in chronically transfused patients with thalassemia, sickle cell anemia, or myelodysplastic syndrome. PLoS ONE |
| title | Cardiac iron overload in chronically transfused patients with thalassemia, sickle cell anemia, or myelodysplastic syndrome. |
| title_full | Cardiac iron overload in chronically transfused patients with thalassemia, sickle cell anemia, or myelodysplastic syndrome. |
| title_fullStr | Cardiac iron overload in chronically transfused patients with thalassemia, sickle cell anemia, or myelodysplastic syndrome. |
| title_full_unstemmed | Cardiac iron overload in chronically transfused patients with thalassemia, sickle cell anemia, or myelodysplastic syndrome. |
| title_short | Cardiac iron overload in chronically transfused patients with thalassemia, sickle cell anemia, or myelodysplastic syndrome. |
| title_sort | cardiac iron overload in chronically transfused patients with thalassemia sickle cell anemia or myelodysplastic syndrome |
| url | http://europepmc.org/articles/PMC5336214?pdf=render |
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