Pesquisa | SciELO

Abstract To date, hydroxyurea is the only effective and safe drug that significantly reduces morbidity and mortality of individuals with Sickle cell disease. Twenty years of real-life experience has demonstrated that hydroxyurea reduces pain attacks, vaso-occlusive events, including acute chest syndrome, the number and duration of hospitalizations and the need for transfusion. The therapeutic success of hydroxyurea is directly linked to access to the drug, the dose used and adherence to treatment which, in part, is correlated to the availability of hydroxyurea. This consensus aims to reduce the number of mandatory exams needed to access the drug, prioritizing the requesting physician's report, without affecting patient safety. date disease reallife real life attacks vasoocclusive vaso occlusive events syndrome transfusion which part physicians physician s report safety

https://doi.org/10.1016/j.htct.2024.01.001

Cardiac iron overload evaluation in thalassaemic patients using T2* magnetic resonance imaging following chelation therapy: a multicentre cross-sectional study T2 T therapy crosssectional cross sectional
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Chapchap, Eduardo Cerello

; Silva, Murilo Marques Almeida

; Assis, Reijane Alves de

; 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

Hematology, Transfusion and Cell Therapy

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Mar 2023, Volume 45 Nº 1 Páginas 7 - 15

Resumo: En | Texto: En | PDF: En

Abstract 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. (MRI T2 T investigation T2R2 TR R2 R T2*/R2 realworld real world thalassemia chelationtransfusion transfusion followup 20042011. 20042011 2004 2011. 2011 2004-2011 13 92 1 2033ngml ngml 2 033ng ml ng range (range 5914,123. 5914123 59 14,123 . 14 123 59-14,123) 99%, 99 99% , (99%) 74% 74 (74% 36%. 36 36% (36%) 12 1. followup, up, 263 63 2,6 mgg mg g 205 05 2,0 p 0.003. 0003 0.003 0 003 0.003) cutoff 78 73 specificity However 5 122 ng/ml NPV (NPV 98 28 assessment evaluation moderatesevere moderate severe T2R T2*/R 2004201 200 201 2004-201 9 5914 5914,123 591412 14123 14,12 59-14,123 (99% (74 3 (36% 26 6 2, 20 000 0.00 00 200420 2004-20 591 5914,12 59141 1412 14,1 59-14,12 (99 (7 (36 0.0 20042 2004-2 5914,1 141 14, 59-14,1 (9 ( (3 0. 2004- 5914, 59-14, 59-14 59-1 59-

https://doi.org/10.1016/j.htct.2021.01.014

Collaboration for success: the Global Initiative for Childhood Cancer in Latin America
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Vásquez, Liliana

; Fuentes-Alabi, Soad

; Benitez-Majano, Sara

; Ribeiro, Karina Braga

; Abraham, Monnie

; Agulnik, Asya

; Baker, Justin N.

; Blanco, Daniel Bastardo

; Caniza, Miguela A.

; Cardenas-Aguirre, Adolfo

; Salaverria, Carmen

; Sullivan, Courtney E.

; Damasco-Avila, Erika

; García-Quintero, Ximena

; Loggetto, Patricia

; McNeil, Michael J.

; Luna-Fineman, Sandra

; Rossell, Nuria

; Garcia de Lima, Regina Aparecida

; de Mendonca, Regina Holanda

; Trigoso, Viviana

; Segovia, Lorena

; Vasquez, Roberto

; Moreno, Florencia

; Friedrich, Paola

; Luciani, Silvana

; Lam, Catherine

; Metzger, Monika L.

; Rodríguez-Galindo, Carlos

; Maza, Mauricio

Revista Panamericana de Salud Pública

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2023, Volume 47 elocation e144

Resumo: Pt Es En | Texto: Pt Es En | PDF: Pt | PDF: Es | PDF: En

RESUMO A Iniciativa Global para o Câncer Infantil tem como objetivo aumentar a taxa de cura de crianças com câncer no mundo todo, melhorando o acesso a cuidados e a qualidade da assistência médica. A Organização Pan-Americana da Saúde (OPAS), o St. Jude Children's Research Hospital (St. Jude) e colaboradores uniram esforços para melhorar o desfecho de crianças com câncer na América Latina e no Caribe (ALC) no âmbito do marco CureAll. Neste artigo, descrevemos o processo de desenvolvimento de recursos regionais com o objetivo de acelerar a implementação da Iniciativa na ALC. Em março de 2021, a OPAS formou grupos de trabalho regionais para desenvolver projetos centrais alinhados com os pilares e facilitadores do CureAll. A partir das reuniões de diálogo regionais, foram criados sete grupos de trabalho: detecção precoce, enfermagem, atenção psicossocial, nutrição, cuidados de suporte, abandono do tratamento e cuidados paliativos. A OPAS organizou reuniões virtuais regulares sob a orientação e o apoio dos programas regionais e transversais do St. Jude e de mentores internacionais. Entre abril e dezembro de 2021, 202 especialistas multidisciplinares participaram de 43 reuniões virtuais para promover o diálogo entre as partes interessadas a fim de melhorar os desfechos do câncer infantil. Foram produzidos 14 materiais técnicos: quatro panoramas regionais, quatro documentos técnicos, dois cursos virtuais, um conjunto de perfis epidemiológicos nacionais, uma série de conteúdo educacional para pais e cuidadores e duas campanhas de comunicação. O diálogo e o compromisso contínuos da OPAS, do St. Jude, dos comitês de trabalho da ALC e dos colaboradores internacionais são bases essenciais para acelerar com sucesso a implementação da Iniciativa Global para o Câncer Infantil. Isso é possível por meio do desenvolvimento de materiais de relevância regional e mundial. São necessárias mais pesquisas e avaliações para determinar o impacto dessas estratégias e recursos nos resultados do câncer infantil na ALC e em outras regiões.

RESUMEN La Iniciativa Mundial contra el Cáncer Infantil tiene como objetivo aumentar a nivel mundial la tasa de curación del cáncer infantil mediante la mejora del acceso a la atención de salud y de su calidad. La Organización Panamericana de la Salud (OPS), el St. Jude Children’s Research Hospital y los colaboradores han aunado esfuerzos para mejorar los resultados en la población infantil con cáncer en América Latina y el Caribe valiéndose del marco CureAll. En este artículo describimos el proceso de elaboración de recursos regionales destinados a acelerar la aplicación de la Iniciativa Mundial en América Latina y el Caribe. En marzo del 2021, la OPS formó grupos de trabajo regionales para elaborar proyectos básicos que estuvieran en consonancia con los pilares y los elementos facilitadores del CureAll. De los diálogos regionales surgieron siete grupos de trabajo: detección temprana, enfermería, aspectos psicosociales, nutrición, tratamientos de apoyo, abandono del tratamiento y cuidados paliativos. La OPS organizó con regularidad reuniones virtuales en las que se contó con la tutoría y el apoyo de programas regionales o transversales del St. Jude Children’s Research Hospital y de mentores internacionales. Entre abril y diciembre del 2021 hubo 43 reuniones virtuales a las que asistieron 202 expertos multidisciplinarios, con el objetivo de promover el diálogo entre las partes interesadas para mejorar los resultados en materia de cáncer infantil. Se elaboraron catorce productos técnicos: cuatro panoramas regionales, cuatro documentos técnicos, dos cursos virtuales, un conjunto de perfiles epidemiológicos de países, una serie con contenidos educativos para padres y cuidadores y dos campañas de comunicación. El diálogo y el compromiso constantes de la OPS, el St. Jude Children’s Research Hospital, los comités de trabajo de América Latina y el Caribe y los colaboradores internacionales son las bases fundamentales para conseguir que se acelere la aplicación de la Iniciativa Mundial. Esto se puede lograr mediante la elaboración de materiales que resulten pertinentes a nivel regional y mundial. Son necesarias más investigaciones y evaluaciones para determinar el impacto que tienen estas estrategias y recursos en los resultados que se obtienen en el cáncer infantil en América Latina y el Caribe y en otras subregiones.

ABSTRACT The Global Initiative for Childhood Cancer (GICC) aims to increase the cure rate for children with cancer globally by improving healthcare access and quality. The Pan American Health Organization (PAHO), St. Jude Children’s Research Hospital (St. Jude), and collaborators have joined efforts to improve outcomes of children with cancer in Latin America and the Caribbean (LAC) using the CureAll framework. In this article, we describe the process of developing regional resources aimed at accelerating the GICC implementation in LAC. In March 2021, PAHO formed regional working groups to develop core projects aligned with CureAll pillars and enablers. Seven working groups emerged from regional dialogues: early detection, nursing, psychosocial, nutrition, supportive care, treatment abandonment, and palliative care. PAHO arranged regular online meetings under the mentorship and support of St. Jude regional/transversal programs and international mentors. Between April and December 2021, 202 multidisciplinary experts attended 43 online meetings to promote the dialogue between stakeholders to improve childhood cancer outcomes. Fourteen technical outputs were produced: four regional snapshots, four technical documents, two virtual courses, one set of epidemiological country profiles, one educational content series for parents/caregivers, and two communication campaigns. The ongoing dialogue and commitment of PAHO, St. Jude, LAC working committees, and international collaborators are essential foundations to successfully accelerate GICC implementation. This is achievable through the development of materials of regional and global relevance. Further research and evaluation are needed to determine the impact of these strategies and resources on childhood cancer outcomes in LAC and other regions.

https://doi.org/10.26633/rpsp.2023.144

Overview of lymphoma diagnosis in Brazilian public health system patients: Open data analysis for health care planning
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Martins, Denise Pires

; Correa-Netto, Nelson Francisco

; Melo, Nina

; Loggetto, Sandra Regina

; de Liberal, Marcia Mello Costa

Hematology, Transfusion and Cell Therapy

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Jan 2022, Volume 44 Nº 1 Páginas 40 - 48

Resumo: En | Texto: En | PDF: En

Abstract Introduction Knowing the information regarding the panorama of lymphoma diagnosis in patients treated in the Brazilian Public Unified Health System from the last 10 years is a challenge for Strategic Health Planning. Objective To characterize the Brazilian population with lymphoma treated in the Brazilian Public Unified Health System between 2008 and 2017 regarding staging, sex, residence site and mortality. Material and methods A descriptive, retrospective, and longitudinal trial with secondary data from DataSUS (SIA/SUS and SIM/SUS) obtained from patients with ICD-10 C81-85. Results There were 70,850 lymphoma cases between 2008 and 2017, of which 55% were male, the median age was 51 years, and 27% had Hodgkin Lymphoma. Most patients (56%) were treated outside the residence city. São Paulo State accounted for 25% of patients. Treatment initiation took more than 60 days in 27% of cases. A total of 45,601 deaths were due to lymphoma (12% Hodgkin Lymphoma and 88% Non-Hodgkin Lymphoma), with a median age 63 years, and were mainly males (55%). Staging data were inadequate in 23% of patients, and analysis was performed only on the valid records. Advanced disease was diagnosed in 58% of patients (60% male; 57% female) and was more common in Non-Hodgkin Lymphoma (62%) versus Hodgkin Lymphoma (49%). Discussion Late diagnosis interferes with mortality rates. Health promotion and cancer prevention campaigns, especially targeting the male public, and training for early diagnosis and early treatment are needed. Conclusion Effective measures for early diagnosis and treatment are urgently needed for lymphoma control.

https://doi.org/10.1016/j.htct.2020.08.017

Guidelines on sickle cell disease: primary stroke prevention in children and adolescents. Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular Guidelines project: Associação Médica Brasileira - 2021
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Loggetto, Sandra Regina

; Veríssimo, Mônica Pinheiro de Almeida

; Darrigo-Junior, Luiz Guilherme

; Simões, Ricardo dos Santos

; Bernardo, Wanderley Marques

; Braga, Josefina Aparecida Pellegrini

Hematology, Transfusion and Cell Therapy

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Jan 2022, Volume 44 Nº 1 Páginas 85 - 94

Resumo: En | Texto: En | PDF: En

https://doi.org/10.1016/j.htct.2021.09.013

Outcome of SARS-CoV-2 and immune thrombocytopenia in a pediatric patient
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Soares, Ana Cláudia Carramaschi Villela

; Loggetto, Sandra Regina

; Manga, Fernanda Cristina Marinho

; Faustino, Laira Rita

; Braga, Josefina Aparecida Pellegrini

Hematology, Transfusion and Cell Therapy

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Mar 2021, Volume 43 Nº 1 Páginas 101 - 103

Resumo: En | Texto: En | PDF: En

https://doi.org/10.1016/j.htct.2020.09.145 181 downloads

Hemoglobinopathy and pediatrics in the time of COVID-19
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Vilela, Thiago de Souza

; Braga, Josefina Aparecida Pellegrini

; Loggetto, Sandra Regina

Hematology, Transfusion and Cell Therapy

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Mar 2021, Volume 43 Nº 1 Páginas 87 - 100

Resumo: En | Texto: En | PDF: En

ABSTRACT Introduction: It is important to know if patients with hemoglobinopathy could be more susceptible to COVID-19. Objective: Analyze SARS-CoV-2 infection in pediatric patients with hemoglobinopathy. Methods: Using the online platforms LILACS, PUBMED and EMBASE, on 17- JUL-2020 a search was made for the terms COVID-19 and SARS-CoV-2 associated with "sickle cell", "thalassemia" and "hemoglobinopathy". Results: There were 623 pediatric and adult patients with sickle cell disease (SCD) or beta thalassemia (BT) and COVID-19. Total mortality rate was 6.42%. No pediatric patient with BT has been described. So, our analysis focused on children and adolescents with SCD: there were 121 pediatric patients, one adolescent died, prophylactic anticoagulation was prescribed to six patients, 11.76% needed intensive care unit, blood transfusion was prescribed in 29.70%. Vaso-occlusive crisis (VOC) and acute chest syndrome (ACS) were the main clinical manifestations in SCD. Discussion: Pediatric patients with SCD and COVID-19 have a low mortality rate when compared to adults, although is higher than the global pediatric population with COVID-19 (0−0.67%). The comorbidities associated with age and the long-term complications inherent to hemoglobinopathies may contribute to the increased mortality outside the pediatric age group. In SCD the clinical manifestations, both in children and adults, are VOC and ACS, and there was increase in blood requirement. Pediatric SCD patients with COVID-19 need more intensive care unit than the global pediatric population (3.30%). Conclusion: Despite pediatric population with SCD needs more intensive care, the outcome after infection by COVID-19 is favorable.

https://doi.org/10.1016/j.htct.2020.11.002 387 downloads

Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular Consensus on genetically modified cells. II: CAR-T cell therapy for patients with CD19+ acute lymphoblastic leukemia
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Seber, Adriana

; Castro Junior, Claudio Galvãode de

; Kerbauy, Lucila N

; Hirayama, Alexandre V

; Bonfim, Carmem

; Fernandes, Juliana Folloni

; Souza, Mair

; Schafell, Rony

; Nabhan, Samir

; Loggetto, Sandra Regina

; Simões, Belinda Pinto

; Rocha, Vanderson

; Lima, Marcos de

; Guerino-Cunha, Renato L

; Bittencourt, Henrique

Hematology, Transfusion and Cell Therapy

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2021, Volume 43 Supl. 2 Páginas 13 - 21

Resumo: En | Texto: En | PDF: En

ABSTRACT Chimeric antigen receptor T (CAR-T) cell therapy is a novel therapeutic modality for acute lymphoblastic leukemia (ALL) with robust outcomes in patients with refractory or relapsed disease. At the same time, CAR-T cell therapy is associated with unique and potentially fatal toxicities, such as cytokine release syndrome (CRS) and neurological toxicities (ICANS). This manuscript aims to provide a consensus of specialists in the fields of Hema- tology Oncology and Cellular Therapy to make recommendations on the current scenario of the use of CAR-T cells in patients with ALL.

https://doi.org/10.1016/j.htct.2021.09.002

Guidelines on Beta-thalassemia major - regular blood transfusion therapy: Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular: project guidelines: Associação Médica Brasileira - 2016
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Langhi Jr, Dante

; Ubiali, Eugênia Maria Amorim

; Marques Jr, José Francisco Comenalli

; Verissimo, Mônica de Almeida

; Loggetto, Sandra Regina

; Silvinato, Antonio

; Bernardo, Wanderley Marques

Revista Brasileira de Hematologia e Hemoterapia

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Dez 2016, Volume 38 Nº 4 Páginas 341 - 345

Resumo: En | Texto: En | PDF: En

https://doi.org/10.1016/j.bjhh.2016.09.003 2173 downloads

10.

Guidelines on neonatal screening and painful vaso-occlusive crisis in sickle cell disease: Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular: Project guidelines: Associação Médica Brasileira - 2016
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Braga, Josefina Aparecida Pellegrini

; Veríssimo, Mônica Pinheiro de Almeida

; Saad, Sara Teresinha Olalla

; Cançado, Rodolfo Delfini

; Loggetto, Sandra Regina

Revista Brasileira de Hematologia e Hemoterapia

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Jun 2016, Volume 38 Nº 2 Páginas 147 - 157

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https://doi.org/10.1016/j.bjhh.2016.04.001 2165 downloads

11.

Sickle cell anemia: clinical diversity and beta S-globin haplotypes
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Loggetto, Sandra Regina

Revista Brasileira de Hematologia e Hemoterapia

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Jun 2013, Volume 35 Nº 3 Páginas 155 - 157

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https://doi.org/10.5581/1516-8484.20130048 2384 downloads

12.

Guidelines on the diagnosis of primary immune thrombocytopenia in children and adolescents: Associacao Brasileira de Hematologia, Hemoterapia e Terapia Celular Guidelines Project: Associacao Medica Brasileira - 2012
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Braga, Josefina Aparecida Pellegrini

; Loggetto, Sandra Regina

; Hoepers, Andrea Thives de Carvalho

; Bernardo, Wanderley Marques

; Medeiros, Leticia

; Verissimo, Monica Pinheiro de Almeida

Revista Brasileira de Hematologia e Hemoterapia

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2013, Volume 35 Nº 5 Páginas 358 - 365

Resumo: En | Texto: En | PDF: En

https://doi.org/10.5581/1516-8484.20130105 3115 downloads

13.

Brazilian Thalassemia Association protocol for iron chelation therapy in patients under regular transfusion
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Verissimo, Monica Pinheiro de Almeida

; Loggetto, Sandra Regina

; Fabron Junior, Antonio

; Baldanzi, Giorgio Roberto

; Hamerschlak, Nelson

; Fernandes, Juliano Lara

; Araujo, Aderson da Silva

; Lobo, Clarisse Lopes de Castro

; Fertrin, Kleber Yotsumoto

; Berdoukas, Vasilios Antonios

; Galanello, Renzo

Revista Brasileira de Hematologia e Hemoterapia

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2013, Volume 35 Nº 6 Páginas 428 - 434

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In the absence of an iron chelating agent, patients with beta-thalassemia on regular transfusions present complications of transfusion-related iron overload. Without iron chelation therapy, heart disease is the major cause of death; however, hepatic and endocrine complications also occur. Currently there are three iron chelating agents available for continuous use in patients with thalassemia on regular transfusions (desferrioxamine, deferiprone, and deferasirox) providing good results in reducing cardiac, hepatic and endocrine toxicity. These practice guidelines, prepared by the Scientific Committee of Associação Brasileira de Thalassemia (ABRASTA), presents a review of the literature regarding iron overload assessment (by imaging and laboratory exams) and the role of T2* magnetic resonance imaging (MRI) to control iron overload and iron chelation therapy, with evidence-based recommendations for each clinical situation. Based on this review, the authors propose an iron chelation protocol for patients with thalassemia under regular transfusions.

https://doi.org/10.5581/1516-8484.20130106 5074 downloads

14.

Guidelines on the treatment of primary immune thrombocytopenia in children and adolescents: Associacao Brasileira de Hematologia, Hemoterapia e Terapia Celular Guidelines Project: Associacao Medica Brasileira - 2012
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Loggetto, Sandra Regina

; Braga, Josefina Aparecida Pellegrini

; Verissimo, Monica Pinheiro de Almeida

; Bernardo, Wanderley Marques

; Medeiros, Leticia

; Hoepers, Andrea Thives de Carvalho

Revista Brasileira de Hematologia e Hemoterapia

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2013, Volume 35 Nº 6 Páginas 417 - 427

Resumo: En | Texto: En | PDF: En

https://doi.org/10.5581/1516-8484.20130124 5159 downloads Citado 4 vezes em SciELO

15.

Iron overload in Brazilian thalassemic patients
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Assis, Reijane Alves de

; Kay, Fernando Uliana

; Rosemberg, Laércio Alberto

; Parma, Alexandre Henrique C.

; Nomura, Cesar Higa

; Loggetto, Sandra Regina

; Araujo, Aderson da Silva

; Fabron Junior, Antonio

; Veríssimo, Mônica Pinheiro de Almeida

; Baldanzi, Giorgio Roberto

; Steagal, Merula A.

; Velloso, Claudia Angela Galleni Di Sessa

; Espósito, Breno Pannia

; Nakashima, Sandra Saemi

; Diniz, Michelli da Silva

; Tricta, Fernando

; Baroni, Ronaldo Hueb

; Funari, Marcelo Buarque de Gusmão

; Wood, John C.

; Ribeiro, Andreza Alice Feitosa

; Hamerschlak, Nelson

einstein (São Paulo)

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Jun 2011, Volume 9 Nº 2 Páginas 165 - 172

Resumo: Pt En | Texto: Pt En | PDF: Pt | PDF: En

Objetivo: Avaliar o acúmulo de ferro em diferentes órgãos por meio da ressonância nuclear magnética T2* e correlacionar os resultados aos níveis de ferritina sérica, ferro plasmático lábil e outros órgãos envolvidos. Métodos: Foram avaliados retrospectivamente 115 pacientes talassêmicos (sendo 65 mulheres). A concentração hepática de ferro foi determinada em biópsia de 11 pacientes; os resultados foram comparados com os valores de T2* fígado. Resultados: a ferritina sérica média foi de 2.676,5 +/- 2.051,7 ng/mL. O ferro plasmático lábil foi anormal (> 0,6 Unidades/mL) em 48/83 pacientes (57%). A média dos valores de T2* no fígado foi 3,91 ± 3,95 ms, sugerindo siderose hepática em 92,1% pacientes. A média do T2* cardíaco foi de 24,96 ± 14,17 ms e 36% dos pacientes apresentavam siderose cardíaca (T2* < 20ms), dos quais 19% (22/115) já apresentavam sobrecarga cardíaca grave (T2* < 10 ms). A média de T2* no pâncreas foi de 11,12 ± 11,20 ms, perfazendo um total de 83,5% de pacientes com sobrecarga de ferro pancreático (T2* < 21 ms). Houve correlação significativa, curvilínea e inversa entre T2* fígado e a concentração de ferro hepática (r = −0,878; p <0,001) e correlação moderada entre T2* pâncreas e T2* miocárdio (r = 0,546; p <0,0001). Conclusão: Uma elevada taxa de acometimento hepático, pancreático e cardíaco por sobrecarga férrica foi demonstrada. Os níveis de ferritina não puderam prever sobrecarga de ferro hepático, cardíaco ou pancreáticos medidos por meio da ressonância nuclear magnética T2*. Não houve correlação entre a sobrecarga de ferro no fígado, pâncreas e miocárdio, nem entre a ferritina e os níveis plasmáticos de ferro sérico e os valores de T2* no fígado, coração e pâncreas.

ABSTRACT Objectives: To evaluate the use of magnetic resonance imaging in patients with β-thalassemia and to compare T2* magnetic resonance imaging results with serum ferritin levels and the redox active fraction of labile plasma iron. Methods: We have retrospectively evaluated 115 chronically transfused patients (65 women). We tested serum ferritin with chemiluminescence, fraction of labile plasma iron by cellular fluorescence and used T2* MRI to assess iron content in the heart, liver, and pancreas. Hepatic iron concentration was determined in liver biopsies of 11 patients and the results were compared with liver T2* magnetic resonance imaging. Results: The mean serum ferritin was 2,676.5 +/- 2,051.7 ng/mL. A fraction of labile plasma iron was abnormal (> 0,6 Units/mL) in 48/83 patients (57%). The mean liver T2* value was 3.91 ± 3.95 ms, suggesting liver siderosis in most patients (92.1%). The mean myocardial T2* value was 24.96 ± 14.17 ms and the incidence of cardiac siderosis (T2* < 20 ms) was 36%, of which 19% (22/115) were severe cases (T2* < 10 ms). The mean pancreas T2* value was 11.12 ± 11.20 ms, and 83.5% of patients had pancreatic iron deposition (T2* < 21 ms). There was significant curvilinear and inverse correlation between liver T2* magnetic resonance imaging and hepatic iron concentration (r= −0.878; p < 0.001) and moderate correlation between pancreas and myocardial T2* MRI (r = 0.546; p < 0.0001). Conclusion: A high rate of hepatic, pancreatic and cardiac impairment by iron overload was demonstrated. Ferritin levels could not predict liver, heart or pancreas iron overload as measured by T2* magnetic resonance imaging. There was no correlation between liver, pancreas, liver and myocardial iron overload, neither between ferritin and fraction of labile plasma iron with liver, heart and pancreas T2* values

https://doi.org/10.1590/s1679-45082011ao1897 3301 downloads

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