HEMATOLOGY AND BLOOD TRANSFUSION DIVISION / DEPARTMENT OF CLINICAL AND EXPERIMENTAL ONCOLOGY ESCOLA PAULISTA DE MEDICINA - UNIVERSIDADE FEDERAL DE SÃO PAULO
Hematological ratios and cytokine profiles in heterozygous beta-thalassemia Ana Carolina Marques Ciceri, Laura Eduarda de Oliveira, Ana Luísa Richter, José Antonio Mainardi de Carvalho, Maylla Rodrigues Lucena, Guilherme Wataru Gomes, Maria Stella Figueiredo, Magnun Nueldo Nunes dos Santos, Vera Lúcia Nascimento Blaia-D'Avila, Rodolfo Delfini Cançado, Elvira Maria Guerra-Shinohara, Clóvis Paniz Hematology Transfusion and Cell Therapy, 2025 INTRODUCTION: β-Thalassemia is defined by a reduced or complete absence of β-globin chain synthesis in hemoglobin, leading to hemolytic anemia. Heterozygous β-thalassemia, also known as β-thalassemia trait (hBTh), the mildest form of this anemia, typically does not cause symptoms in carriers. However, it may lead to changes in the immune system, including an increase in total leukocyte, neutrophil, and lymphocyte counts. OBJECTIVE: This study aimed to evaluate various immune and inflammation markers, including neutrophil/lymphocyte, derived neutrophil/lymphocyte, lymphocyte/monocyte, platelet/lymphocyte, neutrophil/platelet ratios, systemic immune-inflammation index, systemic inflammation response index, neutrophil/natural killer cell ratio (NNKR), and inflammatory cytokines in β-thalassemia trait carriers. METHOD: A retrospective observational study was conducted, including 50 β-thalassemia trait individuals and 100 healthy controls. RESULTS: Leukocyte, neutrophil and reticulocyte counts, and interleukin 6 levels were higher in carriers compared to controls. Notably, the β-thalassemia trait group had increased neutrophil/platelet, neutrophil/lymphocyte and derived neutrophil/lymphocyte ratios, and the systemic immune-inflammation and systemic inflammation response indexes were higher compared to the controls. CONCLUSIONS: β-thalassemia trait shows a more pronounced inflammatory profile as indicated by hematological ratios. These ratios, therefore are potentially cost-effective and easily applicable markers for monitoring patients with the β-thalassemia trait.
Patient Blood Management in Cardiovascular Surgery Isabel Cristina Céspedes, Maria Stella Figueiredo, Antonio Alceu Dos Santos, Nelson A Hossne Brazilian Journal of Cardiovascular Surgery, 2024 Patient Blood Management in
Sickle Cell Disease in Brazil: Current Management Aderson da Silva Araújo, Ana Cristina Silva Pinto, Clarisse Lopes de Castro Lobo, Maria Stella Figueiredo, Sandra Fátima Menosi Gualandro, Sara Teresinha Olalla Saad, Rodolfo Delfini Cançado Hemoglobin, 2024 Sickle cell disease (SCD) comprises inherited red blood cell disorders due to a mutation in the β-globin gene (c20A > T, pGlu6Val) and is characterized by the presence of abnormal hemoglobin, hemoglobin S, hemolysis, and vaso-occlusion. This mutation, either in a homozygous configuration or in compound states with other β-globin mutations, leads to polymerization of hemoglobin S in deoxygenated conditions, causing modifications in red blood cell shape, particularly sickling. Vaso-occlusive crisis (VOC) is the hallmark of the disease, but other severe complications may arise from repeated bouts of VOCs. SCD is considered a global health problem, and its incidence has increased in some areas of the world, particularly the Americas and Africa. Management of the disease varies according to the region of the world, mainly due to local resources and socioeconomic status. This review aimed to describe more recent data on SCD regarding available treatment options, especially in Brazil. New treatment options are expected to be available to all patients, particularly crizanlizumab, which is already approved in the country.
Blood concentrations of α-Klotho and FGF-23 exhibit no correlation with bone mineral density in elderly individuals Karina Moura Sawada, Niele Silva de Moraes, Lara Miguel Quirino Araújo, Fernanda Martins Gazoni, Marise Lazaretti-Castro, Maysa Seabra Cendoroglo, John P. Bilezikian, Maria Stella Figueiredo, Fania Cristina dos Santos Einstein Sao Paulo Brazil, 2024 OBJECTIVE To investigating the relationship between α-Klotho and FGF-23 with bone biochemical markers and bone density findings in extremely aged individuals. METHODS A total of 55 individuals with a mean age of 85.6 years were subjected to clinical, biochemical, and bone mineral density analyses and the enzyme-linked immunosorbent assay-based detection of α-Klotho and FGF-23. The mean, standard deviation, median, and interquartile ranges of the sample values were determined, and Spearman's test for association assessments was used for statistical analysis. RESULTS The study participants expressed median FGF-23 and α-Klotho levels of 69.81 RU/mL (51.43 RU/mL) and 733.43 pg/mL (360.83 pg/mL), respectively. The majority of the participants possessed osteopenia (54.5%) and a vitamin D deficiency (57%). The 25-hydroxyvitamin D concentrations ranged between 7.1 and 47.5ng/mL, with a median of 18.1ng/mL. CONCLUSION No substantial associations were discovered between α-Klotho and FGF-23 levels and bone density in the study participants.
Patient Blood Management Program Implementation: Comprehensive Recommendations and Practical Strategies Isabel Cristina Céspedes, Maria Stella Figueiredo, Nelson A Hossne, I. Suriano, Rita de Cássia Rodrigues, M. Barros, Manoel Antonio de Paiva, Fernanda Chohfi Atallah, Bárbara Burza Benini, Adriano Miziara Gonzalez, Fábio Veiga de Castro Sparapani, Newton de Barros, Ieda Aparecida Carneiro, Celina Mayumi Morita Sarto, Caio Sussumu de Macedo Motoyama, Leonardo Sacchi, Victor Piovezan, Simone Luna de Almeida, Laís da Silva Pereira-Rufino, S. Guizilini, I. Rocco, Nacime Salomão Mansur, J. Arakaki, Antonio Alceu Dos Santos, Carlos E Panfilio Brazilian Journal of Cardiovascular Surgery, 2024 Introduction Blood transfusion is one of the most common medical practices worldwide. However, current scientific literature has shown that the immunomodulatory effects of blood transfusion are associated with an increased likelihood of infection, prolonged hospitalization, and morbimortality. Also, it means high costs for healthcare systems. Methods In this context, acknowledging that blood transfusions are essentially heterologous cell transplantations, the use of therapeutic options has gained strength and is collectively known as the patient blood management (PBM) program. PBM is an approach based on three main pillars: (1) treating anemias and coagulopathies in an optimized manner, especially in the preoperative period; (2) optimizing perioperative hemostasis and the use of blood recovery systems to avoid the loss of the patient's blood; (3) anemia tolerance, with improved oxygen delivery and reduced oxygen demand, particularly in the postoperative period. Results Current scientific evidence supports the effectiveness of PBM by reducing the need for blood transfusions, decreasing associated complications, and promoting more efficient and safer blood management. Thus, PBM not only improves clinical outcomes for patients but also contributes to the economic sustainability of healthcare systems. Conclusion The aim of this review was to summarize PBM strategies in a comprehensive, evidence-based approach through a systematic and structured model for PBM implementation in tertiary hospitals. The recommendations proposed herein are from researchers and experts of a high-complexity university hospital in the network of the Sistema Único de Saúde, presenting itself as a strategy that can be followed as a guideline for PBM implementation in other settings.
Consensus of the Brazilian Association of Hematology, Hemotherapy and Cellular Therapy (ABHH) and the Brazilian Ministry of Health - General management of blood and blood products on the tests necessary for the release of exceptional medicines for sickle cell disease Clarisse Lobo, Aderson Araújo, Alexandre de Albuquerque Antunes, Ana Cristina Silva Pinto, Ariadne Carvalho Godinho, Cassia Silvestre Mariano Pires, Cinthia Cristina Matheus, Xerez de Albuquerque, Daniele Campos Fontes Neves, Fábio de Lima Moreno, Giorgio Baldanzi, Grazziella Curado Siufi, Heloisa Helena Pereira Miranda, Jane Hankins, Joice Aragão, Josefina Aparecida Pellegrini Braga, Juliana Touguinha Neves Martins, Luciana Campos Costa Machado de Souza, Maria Stella Figueiredo, Mirella Rodrigues Oliveira, Patricia Santos Resende Cardoso, Patricia Costa Alves Pinto, Patricia Gomes Moura, Rodolfo Delfini Cançado, Paulo Ivo Cortez de Araujo, Sara Olalla Saad, Sandra Regina Loggetto, Teresa Cristina Cardoso Fonseca Hematology Transfusion and Cell Therapy, 2024 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.
Factors related to the readiness of Brazilian chronic pediatric patients to transition to care in adult clinics Fernanda Souza Angotti Carrara, Daniela Gerent Petry Piotto, Ilana Izidoro Silva, Claudio Arnaldo Len, Gleice Clemente Souza Russo, Sonia Mayumi Chiba, Vera Lucia Sdepanian, Josefina Aparecida Pellegrini Braga, Maria Stella Figueiredo, Maria Cristina Andrade, Marta Liliane de Almeida Maia, Ana Lúcia Abreu, Celia Maria Camelo Silva, Maria Teresa Terreri Jornal De Pediatria, 2023 OBJECTIVE: Advances in medicine have increased the life expectancy of pediatric patients with chronic illnesses, and challenges with the guided transition of adolescents and young adults from pediatric clinics to adult clinics have grown. The aim of this study was to better understand readiness and factors related to this transition process in Brazil. METHOD: In this cross-sectional study of 308 patients aged from 16 to 21 years under follow-up in pediatric specialties, the degree of readiness for transition was assessed using the Transition Readiness Assessment Questionnaire (TRAQ) and its domains. Associations with demographic data, clinical data, socio-economic level, medication adherence, family functionality, and parental satisfaction with health care were evaluated. RESULTS: The median TRAQ score was 3.7 (3.2 - 4.2). Better readiness was associated with female patients, socio-economic class A-B, current active employment, higher level of education, not failing any school year, attending medical appointments alone, functional family, and a good knowledge of disease and medications. A low correlation was observed between TRAQ and age. TRAQ presented good internal consistency (alpha-Cronbach 0.86). In the multiple linear regression, TRAQ score showed a significant association with female gender, advanced age, socio-economic class A-B, better knowledge of disease and medications, and independence to attend appointments alone. CONCLUSION: TRAQ instrument can guide healthcare professionals to identify specific areas of approach, in order to support adolescents with chronic disease to set goals for their own personal development and improve their readiness to enter into the adult healthcare system. In this study, some factors were related to better TRAQ scores.
Sickle cell anemia: hierarchical cluster analysis and clinical profile in a cohort in Brazil Valéria de Freitas Dutra, Thais Priscila Biassi, Maria Stella Figueiredo Hematology Transfusion and Cell Therapy, 2023 INTRODUCTION: Sickle cell anemia is a monogenic disorder caused by a mutation in the β-hemoglobin gene, resulting in sickle hemoglobin that can polymerize. Presentation and clinical course have significant inter-individual variability and classifying these patients for severity is a challenge. METHODS: We applied hierarchical clusters with 10 routine laboratory tests to understand if this grouping could be associated with clinical manifestations. We included 145 adult homozygous patients (SS) at an outpatient clinic in a retrospective study. RESULTS: We found five clusters by counting those that had been differentiated by unconjugated bilirubin, reticulocytes, LDH, leukocytes, lymphocytes and monocytes. When comparing groups to clinical findings, the clusters were different only for liver abnormality. Cluster 3 had the lower median of reticulocytes, LDH, leukocytes, lymphocytes and monocytes and a higher percentage of patients under treatment. Clusters 4 and 5 had higher frequencies of liver impairment and higher medians of reticulocytes, LDH, leukocytes, lymphocytes and monocytes. Hemolysis and inflammation seemed to influence the grouping. CONCLUSION: In our study, cluster analysis showed five groups that exhibited different degrees of inflammation and hemolysis. When comparing clinical data, the result was different only for the criteria of liver abnormality.
Zinc in sickle cell disease: A narrative review Carolinne Thaisa de Oliveira Fernandes Miranda, Karina Marques Vermeulen-Serpa, Ana Carolina Cabañas Pedro, José Brandão-Neto, Sancha Helena de Lima Vale, Maria Stella Figueiredo Journal of Trace Elements in Medicine and Biology, 2022
miRNA profile and disease severity in patients with sickle cell anemia Thaís Priscila Biassi, Elvira Maria Guerra-Shinohara, Patrícia Natália Silva Moretti, Valeria de Freitas Dutra, Ana Carolina Cabañas-Pedro, Grazielle Mecabo, Gisele Wally Braga Colleoni, Maria Stella Figueiredo Annals of Hematology, 2022
Consensus statement for diagnosis and treatment of paroxysmal nocturnal haemoglobinuria Rodolfo D. Cançado, Aderson da Silva Araújo, Alex Freire Sandes, Celso Arrais, Clarisse Lopes de Castro Lobo, Maria Stella Figueiredo, Sandra Fátima Menosi Gualandro, Sara Teresinha Olalla Saad, Fernando Ferreira Costa Hematology Transfusion and Cell Therapy, 2021
Serum folate and cytokines in heterozygous β-thalassemia Clóvis Paniz, Maylla Rodrigues Lucena, Juliano Felix Bertinato, Magnun Nueldo Nunes Santos, Guilherme Wataru Gomes, Maria Stella Figueiredo, Maria de Fátima Sonati, Vera Lúcia Nascimento Blaia‐D Avila, Ralph Green, Elvira Maria Guerra‐Shinohara International Journal of Laboratory Hematology, 2020
Daily supplementation with 5 mg of folic acid in Brazilian patients with hereditary spherocytosis Clóvis Paniz, Maylla Rodrigues Lucena, Juliano Felix Bertinato, Felipe Rebello Lourenço, Bruna Cipriano A Barros, Guilherme Wataru Gomes, Maria Stella Figueiredo, Rodolfo Delfini Cançado, Vera Lúcia Nascimento Blaia-D Avila, Christine M Pfeiffer, Zia Fazili, Ralph Green, Valdemir Melechco Carvalho, Elvira Maria Guerra-Shinohara Journal of Investigative Medicine, 2019
A phased SNP-based classification of sickle cell anemia HBB haplotypes Elmutaz M. Shaikho, John J. Farrell, Abdulrahman Alsultan, Hatem Qutub, Amein K. Al-Ali, Maria Stella Figueiredo, David H.K. Chui, Lindsay A. Farrer, George J. Murphy, Gustavo Mostoslavsky, Paola Sebastiani, Martin H. Steinberg BMC Genomics, 2017
A Comprehensive, Ethnically Diverse Library of Sickle Cell Disease-Specific Induced Pluripotent Stem Cells Seonmi Park, Andreia Gianotti-Sommer, Francisco Javier Molina-Estevez, Kim Vanuytsel, Nick Skvir, Amy Leung, Sarah S. Rozelle, Elmutaz Mohammed Shaikho, Isabelle Weir, Zhihua Jiang, Hong-Yuan Luo, David H.K. Chui, Maria Stella Figueiredo, Abdulraham Alsultan, Amein Al-Ali, Paola Sebastiani, Martin H. Steinberg, Gustavo Mostoslavsky, George J. Murphy Stem Cell Reports, 2017
Contraceptive practices in women with sickle-cell disease Natália S. Carvalho, Josefina Pellegrini Braga, Márcia Barbieri, Maria R. Torloni, Maria S. Figueiredo, Cristina A. F. Guazzelli Journal of Obstetrics and Gynaecology, 2017
Physicians' Perception of Sickle-cell Disease Pain Fátima Lucchesi, Maria Stella Figueiredo, Erika B. Mastandrea, James L. Levenson, Wally R. Smith, Alessandro F. Jacinto, Vanessa de A. Citero Journal of the National Medical Association, 2016
Hereditary hemochromatosis: Mutations in genes involved in iron homeostasis in Brazilian patients Paulo C.J.L. Santos, Rodolfo D. Cançado, Alexandre C. Pereira, Isolmar T. Schettert, Renata A.G. Soares, Regina A. Pagliusi, Rosario D.C. Hirata, Mario H. Hirata, Ana C. Teixeira, Maria Stella Figueiredo, Carlos S. Chiattone, Jose E. Krieger, Elvira M. Guerra-Shinohara Blood Cells Molecules and Diseases, 2011
Brazilian guidelines for transcranial doppler in children and adolescents with sickle cell disease Clarisse Lopes de Castro Lobo, Rodolfo Delfini Cançado, Ana Claudia Celestino Bezerra Leite, Ana Claudia Mendonça dos Anjos, Ana Cristina Silva Pinto, Andre Palma da Cunha Matta, Célia Maria Silva, Gisele Sampaio Silva, João Ricardo Friedrisch, Josefina Aparecida Pellegrini Braga, Marcos Christiano Lange, Maria Stella Figueiredo, Marília Álvares Rugani, Orlando Veloso, Patrícia Gomes Moura, Paulo Ivo Cortez, Robert Adams, Sandra Fátima Menosi Gualandro, Shirley Lopes de Castilho, Ursula Thomé, Viviane Flumignan Zetola Revista Brasileira De Hematologia E Hemoterapia, 2011
Hemojuvelin and hepcidin genes sequencing in Brazilian patients with primary iron overload Paulo Caleb Júnior de Lima Santos, Alexandre C. Pereira, Rodolfo D. Cançado, Isolmar T. Schettert, Rosario D.C. Hirata, Mario H. Hirata, Maria Stella Figueiredo, Carlos S. Chiattone, Jose E. Krieger, Elvira M. Guerra-Shinohara Genetic Testing and Molecular Biomarkers, 2010
COVID-19 as a trigger of acute chest syndrome in a pregnant woman with sickle cell anemia CC Justino, FF Campanharo, MN Augusto, SC de Morais, MS Figueiredo Hematology, transfusion and cell therapy 42 (3), 212-214 , 2020 2020 Citations: 28
A daily dose of 5 mg folic acid for 90 days is associated with increased serum unmetabolized folic acid and reduced natural killer cell cytotoxicity in healthy Brazilian adults C Paniz, JF Bertinato, MR Lucena, E De Carli, PM da Silva Amorim, ... The Journal of nutrition 147 (9), 1677-1685 , 2017 2017 Citations: 94
Relationship between serum 25-hydroxyvitamin D and inflammatory cytokines in paediatric sickle cell disease SA Adegoke, OS Smith, AD Adekile, MS Figueiredo Cytokine 96, 87-93 , 2017 2017 Citations: 58
A comprehensive, ethnically diverse library of sickle cell disease-specific induced pluripotent stem cells S Park, A Gianotti-Sommer, FJ Molina-Estevez, K Vanuytsel, N Skvir, ... Stem Cell Reports 8 (4), 1076-1085 , 2017 2017 Citations: 56
The compound state: Hb S/beta-thalassemia MS Figueiredo Revista brasileira de hematologia e hemoterapia 37 (3), 150-152 , 2015 2015 Citations: 52
Hereditary hemochromatosis: mutations in genes involved in iron homeostasis in Brazilian patients PCJL Santos, RD Cançado, AC Pereira, IT Schettert, RAG Soares, ... Blood Cells, Molecules, and Diseases 46 (4), 302-307 , 2011 2011 Citations: 76
Brain magnetic resonance imaging abnormalities in adult patients with sickle cell disease: correlation with transcranial Doppler findings GS Silva, P Vicari, MS Figueiredo, H Carrete, MH Idagawa, AR Massaro Stroke 40 (7), 2408-2412 , 2009 2009 Citations: 65
Clinical, hematological, and molecular characterization of sickle cell anemia pediatric patients from two different cities in Brazil IM Lyra, MS Gonçalves, JAP Braga, MF Gesteira, MH Carvalho, ... Cadernos de saude publica 21 (4), 1287-1290 , 2005 2005 Citations: 72
Renal dysfunction in patients with sickle cell anemia or sickle cell trait R Sesso, MA Almeida, MS Figueiredo, JO Bordin Brazilian journal of medical and biological research 31 (10), 1257-1262 , 1998 1998 Citations: 77
Effect of α‐thalassemia and β‐globin gene cluster haplotypes on the hematological and clinical features of sickle‐cell anemia in Brazil MS Figueiredo, J Kerbauy, MS Gonçalves, VR Arruda, STO Saad, ... American journal of hematology 53 (2), 72-76 , 1996 1996 Citations: 120
MOST CITED SCHOLAR PUBLICATIONS
Effect of α‐thalassemia and β‐globin gene cluster haplotypes on the hematological and clinical features of sickle‐cell anemia in Brazil MS Figueiredo, J Kerbauy, MS Gonçalves, VR Arruda, STO Saad, ... American journal of hematology 53 (2), 72-76 , 1996 1996 Citations: 120
A daily dose of 5 mg folic acid for 90 days is associated with increased serum unmetabolized folic acid and reduced natural killer cell cytotoxicity in healthy Brazilian adults C Paniz, JF Bertinato, MR Lucena, E De Carli, PM da Silva Amorim, ... The Journal of nutrition 147 (9), 1677-1685 , 2017 2017 Citations: 94
Renal dysfunction in patients with sickle cell anemia or sickle cell trait R Sesso, MA Almeida, MS Figueiredo, JO Bordin Brazilian journal of medical and biological research 31 (10), 1257-1262 , 1998 1998 Citations: 77
Hereditary hemochromatosis: mutations in genes involved in iron homeostasis in Brazilian patients PCJL Santos, RD Cançado, AC Pereira, IT Schettert, RAG Soares, ... Blood Cells, Molecules, and Diseases 46 (4), 302-307 , 2011 2011 Citations: 76
Clinical, hematological, and molecular characterization of sickle cell anemia pediatric patients from two different cities in Brazil IM Lyra, MS Gonçalves, JAP Braga, MF Gesteira, MH Carvalho, ... Cadernos de saude publica 21 (4), 1287-1290 , 2005 2005 Citations: 72
Brain magnetic resonance imaging abnormalities in adult patients with sickle cell disease: correlation with transcranial Doppler findings GS Silva, P Vicari, MS Figueiredo, H Carrete, MH Idagawa, AR Massaro Stroke 40 (7), 2408-2412 , 2009 2009 Citations: 65
Relationship between serum 25-hydroxyvitamin D and inflammatory cytokines in paediatric sickle cell disease SA Adegoke, OS Smith, AD Adekile, MS Figueiredo Cytokine 96, 87-93 , 2017 2017 Citations: 58
A comprehensive, ethnically diverse library of sickle cell disease-specific induced pluripotent stem cells S Park, A Gianotti-Sommer, FJ Molina-Estevez, K Vanuytsel, N Skvir, ... Stem Cell Reports 8 (4), 1076-1085 , 2017 2017 Citations: 56
The compound state: Hb S/beta-thalassemia MS Figueiredo Revista brasileira de hematologia e hemoterapia 37 (3), 150-152 , 2015 2015 Citations: 52
COVID-19 as a trigger of acute chest syndrome in a pregnant woman with sickle cell anemia CC Justino, FF Campanharo, MN Augusto, SC de Morais, MS Figueiredo Hematology, transfusion and cell therapy 42 (3), 212-214 , 2020 2020 Citations: 28