Cardiac iron overload evaluation in thalassaemic patients using T2 magnetic resonance imaging following chelation therapy: a multicentre cross-sectional study

Magnetic resonance imaging (MRI) T2* technique is used to assess iron overload in the heart, liver and pancreas of thalassaemic patients. Optimal iron chelation and expected tissue iron response rates remain under investigation. The objective of this study was to analyse serum ferritin and the iron...

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Published in:Hematology, Transfusion and Cell Therapy Transfusion and Cell Therapy, 2023-01, Vol.45 (1), p.7-15
Main Authors: Chapchap, Eduardo Cerello, Silva, Murilo Marques Almeida, de Assis, Reijane Alves, Kerbauy, Lucila Nassif, Diniz, Michelli da Silva, Rosemberg, Laércio Alberto, Loggetto, Sandra Regina, Araujo, Aderson da Silva, Fabron Junior, Antonio, Verissimo, Monica Pinheiro de Almeida, Baldanzi, Giorgio Roberto, Esposito, Breno Pannia, Tricta, Fernando, Steagall, Merula Emmanoel Anargyrou, Vellozo, Claudia Ângela Galleni Di Sessa, Fertrin, Kleber Yotsumoto, Baroni, Ronaldo Hueb, Hamerschlak, Nelson
Format: Article
Language:eng ; por
Subjects:
Chelation therapy
Iron overload
Magnetic resonance imaging
MEDICINE, GENERAL & INTERNAL
Original
Thalassemia
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Summary:Magnetic resonance imaging (MRI) T2* technique is used to assess iron overload in the heart, liver and pancreas of thalassaemic patients. Optimal iron chelation and expected tissue iron response rates remain under investigation. The objective of this study was to analyse serum ferritin and the iron concentration in the heart, liver and pancreas measured by MRI T2*/R2* during regular chelation therapy in a real-world cohort of patients with thalassemia. We evaluated thalassaemic patients ≥ 7 years old undergoing chelation/transfusion therapy by MRI and assessed serum ferritin at baseline and follow-up from 2004-2011. We evaluated 136 patients, 92% major thalassaemic, with a median age of 18 years, and median baseline ferritin 2.033ng/ml (range: 59-14,123). Iron overload distribution was: liver (99%), pancreas (74%) and heart (36%). After a median of 1.2 years of follow-up, the iron overload in the myocardium reduced from 2,63 Fe mg/g to 2,05 (p 0.003). The optimal R2* pancreas cut-off was 148 Hertz, achieving 78% sensitivity and 73% specificity. However, when combining the R2* pancreas cut off ≤ 50 Hertz and a ferritin ≤ 1222 ng/ml, we could reach a negative predictive value (NPV) of 98% for cardiac siderosis. Only 28% were undergoing combined chelation at baseline assessment, which increased up to 50% on follow up evaluation. Chelation therapy significantly reduced cardiac siderosis in thalassaemic patients. In patients with moderate/severe liver iron concentration undergoing chelation therapy, ferritin levels and myocardium iron improved earlier than the liver siderosis.
ISSN:2531-1379
2531-1387
2531-1387
DOI:10.1016/j.htct.2021.01.014