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

Autor: Amandine Baptiste, Thibaud Lefebvre, Olivier Ernst, Emmanuelle Chauzit, Jean-Pierre Vannier, Agnès Guerci-Bresler, Christian Rose, Zoubida Karim, Hervé Puy, Aspasia Stamatoullas, Mariane De Montalembert, Valentine Brousse, Krimo Bouabdallah, Mohamed Touati, C. Dumesnil, Agnès Lahary, Marina Cavazzana, Caroline Elie, Jean-Antoine Ribeil
Přispěvatelé: Service de pédiatrie générale, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Descartes - Paris 5 (UPD5)-Centre de Référence de la Drépanocytose-CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Cytokines, hématopoïèse et réponse immune (CHRI), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Service de pédiatrie générale [CHU Necker], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Service d'Hématologie Clinique [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Service d'Hématologie, Centre de Lutte Contre le Cancer Henri Becquerel Normandie Rouen (CLCC Henri Becquerel), Micro-Environnement et Régulation Cellulaire Intégrée (MERCI), Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Normandie Université (NU), Physiopathologie, Autoimmunité, maladies Neuromusculaires et THErapies Régénératrices (PANTHER), Normandie Université (NU)-Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Rouen, Normandie Université (NU), Hôpital Charles Nicolle [Rouen], Service d'Hématologie biologique [CHU Limoges], CHU Limoges, Hôpital Haut-Lévêque, Université Sciences et Technologies - Bordeaux 1-CHU Bordeaux [Bordeaux], Département de Biothérapie [CHU Necker], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Descartes - Paris 5 (UPD5)-CHU Necker - Enfants Malades [AP-HP], CHU Bordeaux [Bordeaux], Hôpital Cochin [AP-HP], Centre de recherche sur l'Inflammation (CRI (UMR_S_1149 / ERL_8252 / U1149)), Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Service d'informatique médicale et biostatistiques [CHU Necker], Radiologie interventionnelle, Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Service d'hématologie, Hôpital Saint-Vincent-de-Paul, TAN, Yossan-Var, Université Sciences et Technologies - Bordeaux 1 (UB)-CHU Bordeaux [Bordeaux], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Centre de Référence de la Drépanocytose, Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Rouen Normandie (UNIROUEN), CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Descartes - Paris 5 (UPD5), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Paris Diderot - Paris 7 (UPD7), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)
Jazyk: angličtina
Rok vydání: 2017
Předmět:
Male
Blood transfusion
Physiology
Thalassemia
medicine.medical_treatment
[SDV]Life Sciences [q-bio]
lcsh:Medicine
030204 cardiovascular system & hematology
Gastroenterology
Physical Chemistry
Biochemistry
Diagnostic Radiology
0302 clinical medicine
hemic and lymphatic diseases
Medicine and Health Sciences
Erythropoiesis
10. No inequality
Child
lcsh:Science
Multidisciplinary
[SDV.MHEP] Life Sciences [q-bio]/Human health and pathology
biology
Chelation
Radiology and Imaging
Heart
Hematology
Middle Aged
Magnetic Resonance Imaging
Sickle cell anemia
Clinical Laboratory Sciences
3. Good health
[SDV] Life Sciences [q-bio]
Chemistry
Genetic Diseases
030220 oncology & carcinogenesis
Physical Sciences
Female
Anatomy
Research Article
Adult
medicine.medical_specialty
Iron Overload
Adolescent
Anemia
Imaging Techniques
Iron
Anemia
Sickle Cell

Research and Analysis Methods
Iron Chelating Agents
03 medical and health sciences
Autosomal Recessive Diseases
Hepcidin
Diagnostic Medicine
Internal medicine
medicine
Humans
Blood Transfusion
Clinical Genetics
Ferritin
Sickle Cell Disease
Chemical Bonding
business.industry
Transfusion Medicine
Myelodysplastic syndromes
Myocardium
lcsh:R
Biology and Life Sciences
Proteins
Protein Complexes
medicine.disease
Hematopoiesis
Hemoglobinopathies
Myelodysplastic Syndromes
biology.protein
Cardiovascular Anatomy
lcsh:Q
business
Physiological Processes
[SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
Zdroj: PLoS ONE
PLoS ONE, Public Library of Science, 2017, 12 (3), pp.e0172147. ⟨10.1371/journal.pone.0172147⟩
PLoS ONE, 2017, 12 (3), pp.e0172147. ⟨10.1371/journal.pone.0172147⟩
PLoS ONE, Vol 12, Iss 3, p e0172147 (2017)
ISSN: 1932-6203
Popis: 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*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
Databáze: OpenAIRE