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

Introduction: 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...

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Main Authors: Eduardo Cerello Chapchap, Murilo Marques Almeida Silva, Reijane Alves de Assis, Lucila Nassif Kerbauy, Michelli da Silva Diniz, Laércio Alberto Rosemberg, Sandra Regina Loggetto, Aderson da Silva Araujo, Antonio Fabron Junior, Monica Pinheiro de Almeida Verissimo, Giorgio Roberto Baldanzi, Breno Pannia Esposito, Fernando Tricta, Merula Emmanoel Anargyrou Steagall, Claudia Ângela Galleni Di Sessa Vellozo, Kleber Yotsumoto Fertrin, Ronaldo Hueb Baroni, Nelson Hamerschlak
Format: Article
Language:English
Published: Elsevier 2023-01-01
Series:Hematology, Transfusion and Cell Therapy
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S2531137921000432
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author Eduardo Cerello Chapchap
Murilo Marques Almeida Silva
Reijane Alves de Assis
Lucila Nassif Kerbauy
Michelli da Silva Diniz
Laércio Alberto Rosemberg
Sandra Regina Loggetto
Aderson da Silva Araujo
Antonio Fabron Junior
Monica Pinheiro de Almeida Verissimo
Giorgio Roberto Baldanzi
Breno Pannia Esposito
Fernando Tricta
Merula Emmanoel Anargyrou Steagall
Claudia Ângela Galleni Di Sessa Vellozo
Kleber Yotsumoto Fertrin
Ronaldo Hueb Baroni
Nelson Hamerschlak
author_facet Eduardo Cerello Chapchap
Murilo Marques Almeida Silva
Reijane Alves de Assis
Lucila Nassif Kerbauy
Michelli da Silva Diniz
Laércio Alberto Rosemberg
Sandra Regina Loggetto
Aderson da Silva Araujo
Antonio Fabron Junior
Monica Pinheiro de Almeida Verissimo
Giorgio Roberto Baldanzi
Breno Pannia Esposito
Fernando Tricta
Merula Emmanoel Anargyrou Steagall
Claudia Ângela Galleni Di Sessa Vellozo
Kleber Yotsumoto Fertrin
Ronaldo Hueb Baroni
Nelson Hamerschlak
author_sort Eduardo Cerello Chapchap
collection DOAJ
description Introduction: 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. Methods: 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. Results: 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. Conclusions: 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.
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spelling doaj.art-c8ed232d608c4a8084c62faf3ad93cdb2023-02-04T04:18:22ZengElsevierHematology, Transfusion and Cell Therapy2531-13792023-01-01451715Cardiac iron overload evaluation in thalassaemic patients using T2* magnetic resonance imaging following chelation therapy: a multicentre cross-sectional studyEduardo Cerello Chapchap0Murilo Marques Almeida Silva1Reijane Alves de Assis2Lucila Nassif Kerbauy3Michelli da Silva Diniz4Laércio Alberto Rosemberg5Sandra Regina Loggetto6Aderson da Silva Araujo7Antonio Fabron Junior8Monica Pinheiro de Almeida Verissimo9Giorgio Roberto Baldanzi10Breno Pannia Esposito11Fernando Tricta12Merula Emmanoel Anargyrou Steagall13Claudia Ângela Galleni Di Sessa Vellozo14Kleber Yotsumoto Fertrin15Ronaldo Hueb Baroni16Nelson Hamerschlak17Hospital Israelita Albert Einstein, São Paulo, SP, BrazilHospital Israelita Albert Einstein, São Paulo, SP, BrazilFundação de Hematologia e Hemoterapia de Pernambuco (HEMOPE), Recife, PE, BrazilHospital Israelita Albert Einstein, São Paulo, SP, BrazilHospital Israelita Albert Einstein, São Paulo, SP, BrazilHospital Israelita Albert Einstein, São Paulo, SP, BrazilHospital Infantil Sabará, São Paulo, SP, BrazilFundação de Hematologia e Hemoterapia de Pernambuco (HEMOPE), Recife, PE, BrazilHemocentro de Marília, Marília, SP, BrazilCentro de Pesquisa Boldrini Campinas, SP, BrazilCentro de Hematologia e Hemoterapia do Paraná (HEMOPAR), Curitiba, PR, BrazilInstituto de Química, Universidade de São Paulo (USP), São Paulo, SP, BrazilClinical Trials Department Apotex, Toronto, CanadaAssociação Brasileira de Linfoma e Leucemia (ABRALE), São Paulo, SP, BrazilAssociação Brasileira de Linfoma e Leucemia (ABRASTA), São Paulo, SP, BrazilUniversidade de Campinas (UNICAMP), Campinas, SP, BrazilHospital Israelita Albert Einstein, São Paulo, SP, BrazilHospital Israelita Albert Einstein, São Paulo, SP, Brazil; Corresponding author at: Centro de Oncologia e Hematologia Familia Dayan-Daycoval, Hospital Israelita Albert Einstein, Avenida Albert Einstein, 627/701, Bloco A, 3o Subsolo, São Paulo, SP, Brazil, 05651901, Phone: +55-11-2151-1128Introduction: 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. Methods: 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. Results: 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. Conclusions: 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.http://www.sciencedirect.com/science/article/pii/S2531137921000432Iron overloadThalassemiaMagnetic resonance imagingChelation therapy
spellingShingle Eduardo Cerello Chapchap
Murilo Marques Almeida Silva
Reijane Alves de Assis
Lucila Nassif Kerbauy
Michelli da Silva Diniz
Laércio Alberto Rosemberg
Sandra Regina Loggetto
Aderson da Silva Araujo
Antonio Fabron Junior
Monica Pinheiro de Almeida Verissimo
Giorgio Roberto Baldanzi
Breno Pannia Esposito
Fernando Tricta
Merula Emmanoel Anargyrou Steagall
Claudia Ângela Galleni Di Sessa Vellozo
Kleber Yotsumoto Fertrin
Ronaldo Hueb Baroni
Nelson Hamerschlak
Cardiac iron overload evaluation in thalassaemic patients using T2* magnetic resonance imaging following chelation therapy: a multicentre cross-sectional study
Hematology, Transfusion and Cell Therapy
Iron overload
Thalassemia
Magnetic resonance imaging
Chelation therapy
title Cardiac iron overload evaluation in thalassaemic patients using T2* magnetic resonance imaging following chelation therapy: a multicentre cross-sectional study
title_full Cardiac iron overload evaluation in thalassaemic patients using T2* magnetic resonance imaging following chelation therapy: a multicentre cross-sectional study
title_fullStr Cardiac iron overload evaluation in thalassaemic patients using T2* magnetic resonance imaging following chelation therapy: a multicentre cross-sectional study
title_full_unstemmed Cardiac iron overload evaluation in thalassaemic patients using T2* magnetic resonance imaging following chelation therapy: a multicentre cross-sectional study
title_short Cardiac iron overload evaluation in thalassaemic patients using T2* magnetic resonance imaging following chelation therapy: a multicentre cross-sectional study
title_sort cardiac iron overload evaluation in thalassaemic patients using t2 magnetic resonance imaging following chelation therapy a multicentre cross sectional study
topic Iron overload
Thalassemia
Magnetic resonance imaging
Chelation therapy
url http://www.sciencedirect.com/science/article/pii/S2531137921000432
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