Maria Stella Figueiredo
@sp.unifesp.br
HEMATOLOGY AND BLOOD TRANSFUSION DIVISION / DEPARTMENT OF CLINICAL AND EXPERIMENTAL ONCOLOGY
ESCOLA PAULISTA DE MEDICINA - UNIVERSIDADE FEDERAL DE SÃO PAULO
RESEARCH, TEACHING, or OTHER INTERESTS
Hematology, Molecular Biology
Scopus Publications
Scholar Citations
Scholar h-index
Scholar i10-index
Scopus Publications
Jane S Hankins, Clarisse Lobo, Josefina A P Braga, Tarun Aurora, Kelly Pimenta, Maria Stella Figueiredo, and Ana A Baumann
Elsevier BV
Aderson da Silva Araújo, Ana Cristina Silva Pinto, Clarisse Lopes de Castro Lobo, Maria Stella Figueiredo, Sandra Fátima Menosi Gualandro, Sara Teresinha Olalla Saad, and Rodolfo Delfini Cançado
Informa UK Limited
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.
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,et al.
Elsevier BV
Pedro Paulo de Alcantara Pedro, Charlles Heldan de Moura Castro, Marcelo de Medeiros Pinheiro, Lucila Macedo Gonçalves, Maria Stella Figueiredo, and Vera Lúcia Szejnfeld
Wiley
Low bone mass, osteoporosis and
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,et al.
Elsevier BV
Aderson da Silva Araújo, Ana Cristina Silva Pinto, Clarisse Lopes de Castro Lobo, Maria Stella Figueiredo, Sandra Fátima Menosi Gualandro, Sara Teresinha Olalla Saad, and Rodolfo Delfini Cancado
Informa UK Limited
Abstract The polymerization of hemoglobin under deoxygenation is the main pathophysiological event in sickle cell diseases, described more than 70 years ago. The last two decades have seen a major increase in knowledge about the cascade of events that follow the polymerization of hemoglobin and the ensuing sickling of red blood cells. Several distinctive therapeutic targets have been discovered as a result, and a few drugs with innovative mechanisms of action are already on the market, while several others are the focus of ongoing trials. The aim of this narrative review is to describe some of the more recent data in the SCD literature regarding pathophysiology and novel treatments.
Valéria de Freitas Dutra, Thais Priscila Biassi, and Maria Stella Figueiredo
Elsevier BV
Carolinne Thaisa de Oliveira Fernandes Miranda, Karina Marques Vermeulen-Serpa, Ana Carolina Cabañas Pedro, José Brandão-Neto, Sancha Helena de Lima Vale, and Maria Stella Figueiredo
Elsevier BV
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, and Maria Stella Figueiredo
Springer Science and Business Media LLC
Valéria de Freitas Dutra, Vinícius Nunes Cordeiro Leal, Fernanda Pereira Fernandes, Cláudia Regina Lustosa Souza, Maria Stella Figueiredo, and Alessandra Pontillo
Cytokine Elsevier BV
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, and Fernando Ferreira Costa
Hematology, Transfusion and Cell Therapy Elsevier BV
Paroxysmal nocturnal hemoglobinuria is a chronic, multi-systemic, progressive and life-threatening disease characterized by intravascular hemolysis, thrombotic events, serious infections and bone marrow failure. Paroxysmal nocturnal hemoglobinuria results from the expansion of a clone of hematopoietic cells that due to an inactivating mutation of the X-linked gene PIG-A are deficient in glycosylphosphatidylinositol-linked proteins. Early diagnosis, using flow cytometry performed on peripheral blood, the gold standard test to confirm the diagnosis of paroxysmal nocturnal hemoglobinuria, is essential for improved patient management and prognosis. The traditional therapy for paroxysmal nocturnal hemoglobinuria includes blood transfusion, anti-thrombosis prophylaxis or allogeneic bone marrow transplantation. The treatment that has recently become available is the complement blockade by the anti-C5 monoclonal antibody eculizumab. In this consensus, we are aiming to review the diagnosis and treatment of the paroxysmal nocturnal hemoglobinuria patients, as well as the early recognition of its systemic complications. These procedures express the opinions of experts and have been based on the best available evidence and international guidelines, with the purpose of increasing benefits and reducing harm to patients.
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, and Elvira Maria Guerra‐Shinohara
International Journal of Laboratory Hematology Wiley
Suely Roizenblatt, Ana Carolina Cabañas‐Pedro, and Maria Stella Figueiredo
Wiley
SM received research funding from Abbvie, AstraZeneca, BeiGene, Genentech, Gilead, Janssen, Juno, Novartis, Pharmacyclics and TG Therapeutics; and received honorarium for advisory board or lecturing for Abbvie, AstraZeneca, BeiGene, Genentech, Gilead, Janssen, Kite and Pharmacyclics. AB received honorarium for lecturing for Bayer, Foundation Medicine and Pfizer. The other authors declare no conflicts of interest.
Caio Cesar Justino, Felipe Favorette Campanharo, Marina Nobrega Augusto, Stela Cezarino de Morais, and Maria Stella Figueiredo
Elsevier BV
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,et al.
SAGE Publications
Patients with hereditary spherocytosis (HS) have increased rates of erythropoiesis and higher folate requirements. In a case-control study of patients with HS, we evaluated the associations between the use of 5 mg folic acid (FA) daily and serum concentrations of folate, unmetabolized folic acid (UMFA), interleukin (IL)-6, IL-8, IL-10, interferon-γ (IFN-γ) and tumor necrosis factor-α (TNF-α); and mRNA expression of dihydrofolate reductase ( DHFR), methylene tetrahydrofolate reductase ( MTHFR), IL8, IFNG and TNFA genes. Total serum folate and folate forms were measured in 27 patients with HS (21 users [HS-U] and 6 non-users [HS-NU] of supplemental FA) and 54 healthy controls not consuming 5 mg/day supplemental FA. Each patient was matched to two controls based on age, sex and body mass index. The mononuclear leucocyte mRNA expression of relevant genes and their products were determined. Serum folate, UMFA, 5-methyl-tetrahydrofolate (5-methyl-THF) and tetrahydrofolate (THF) concentrations were significantly higher in HS-U compared with matched healthy controls (p<0.001, n=42). HS-NU had lower serum folate concentrations than matched healthy controls (p=0.044, n=12). HS-U and HS-NU presented similar hematological and biochemical markers profiles. No differences were found between HS-U and HS-NU for cytokine serum concentrations and mRNA expression genes. DHFR mRNA expression was higher in HS-U than in HS-NU. The use of high daily doses of FA for treatment of patients with HS may be excessive and is associated with elevated serum UMFA and elevated DHFR mRNA expression. It is not known whether long-term high-dose FA use by patients with HS might have adverse health effects.
Paulo Roberto Juliano Martins, Fernanda Bernadelli De Vito, Gláucia Aparecida Domingos Resende, José Kerbauy, Gilberto de Araújo Pereira, Helio Moraes‐Souza, Maria Stella Figueiredo, and Ieda Therezinha Verreschi
Wiley
AbstractObjectiveInvestigate the gonadal hormonal function in sickle cell individuals.ContextSickle cell disease (SCD) is associated with delayed physical and sexual development, and it has been related to both primary testicular failure and hypothalamo‐pituitary‐gonadal axis abnormalities.DesignThe study of the pituitary gonadotrophin reserve was done evaluating the hormonal levels before and after stimulation by gonadoliberin.PatientsMale patients with homozygous SCD (18‐39 years, median = 29.5 years).MeasurementsGonadal function was evaluated through clinical parameters and the hormonal quantification.ResultsAlthough low body weight and other clinical signs of undernutrition such as clinical hypoandrogenism and the extreme retardation of puberty were seen in these patients, final stature and hormonal testicular reserve to hCG stimulation were proved to be normal according to our previous data. In the present investigation, the basal luteotropic gonadotropin (LH), follicle‐stimulating hormone (FSH) and testosterone (T) levels were similar between the patients and controls. Prostate‐specific antigen (PSA) levels—used as a biochemical marker of androgenicity, mainly in puberty—were lower in the patients than in the controls and were only correlated with T. A subtle abnormality in the pituitary responsivity to gonadotropin‐releasing hormone (GnRH) was disclosed, with a higher response to LH 60 minutes after stimulation in patients than in controls.ConclusionsThese data, in addition to both the clinical and biochemical signs of hypoandrogenism associated with normal to elevated T levels strongly suggest a peripheral origin of hypogonadism, which is probably due to androgen resistance in the patients with SCD.
Bruno Ribeiro Cruz, Thamy Caroline de Souza Silva, Bianca de Souza Castro, Akemi Kuroda Chiba, Elyse Moritz, Josefina Pellegrini Braga, Maria Stella Figueiredo, and José O Bordin
Wiley
Background and ObjectivesThe high homology and the inverted orientation of RHD and RHCE may give rise to non‐functional and aberrant RH alleles. RH genotyping is used to screen RH matched donors to African descent patients. This study aimed to define a strategy for testing RHD and RHCE variants in blood donors to provide compatible units for transfusion of patients with haematological diseases.Materials and MethodsSamples from 132 patients [101 Sickle cell disease (SCD), 14 myelodysplastic syndrome (MDS), 17 acute myelogenous leukaemia (AML)] and 198 Brazilian donors were studied. Major blood group alleles, RHD, RHCE alleles and RHD zygosity were determined by the blood‐MLPA assay. Sequencing was performed to determine RHD and RHCE variant subtypes. A match was an RH genotype that did not encode Rh antigens absent in the patient, along with matching for ABO, MNS, KEL, FY, JK and DI antigens.ResultsOverall, 7·6% of blood donors and 17.4% of patients presented RH genotypes that predict expression of partial Rh antigens or lack of high prevalence Rh antigens. From 23 patients with clinically relevant RH genotypes, 15 had available matched donors.ConclusionWe report the presence of clinically relevant RH genotypes in SCD and in non‐SCD patients. In our admixed population, many patients carry variant RHCE alleles in heterozygosity with normal RHCE alleles. Thus, our results suggest that donors could be selected based on the normal RH allele.
Rejane de Souza Macedo-Campos, Samuel Ademola Adegoke, Maria Stella Figueiredo, Josefina Aparecida Pellegrini Braga, and Gisele Sampaio Silva
Elsevier BV
Samuel Ademola Adegoke, Rejane de Souza Macedo-Campos, Josefina Aparecida Pellegrini Braga, Maria Stella Figueiredo, and Gisele Sampaio Silva
Elsevier BV
Sandra Luzinete Felix de Freitas, Maria Lucia Ivo, Maria Stella Figueiredo, Maria Auxiliadora de Souza Gerk, Cristina Brandt Nunes, and Fernando de Freitas Monteiro
FapUNIFESP (SciELO)
ABSTRACT Objective: To identify the available evidence in the literature on health-related quality of life in adults with sickle cell disease. Method: integrative review of MEDLINE, CUMED, LILACS and SciELO databases, from articles developed in this area, published between 2005 and 2015, in English, Portuguese or Spanish. Results: 22 articles were included, six scales were used to evaluate health-related quality of life scores: three generic and three specific. No specific scale for adults with sickle cell disease has been adapted to Brazilian Portuguese so far. Patients affected by frequent painful crises, with low adherence to treatment, had a compromised quality of life. Conclusion: Selected studies have shown that patients with sickle cell disease have worse scores than the general population. These indicators should be instrumental to the nurse in the proposal of interventions and strategies of assistance and socio-educational, with a view to improving the quality of life of this clientele.
Samuel A. Adegoke, Josefina A.P. Braga, Adekunle D. Adekile, and Maria S. Figueiredo
Ovid Technologies (Wolters Kluwer Health)
Objective: To evaluate the impact of hydroxyurea (HU) on nutritional status and serum 25-hydroxyvitamin D (25-OHD) of children with sickle cell disease (SCD). Design: Anthropometry and serum 25-OHD were determined in 98 children with SCD, comprising of 68 in HU-group and 30 in HU-naive group. Results: Underweight was more common among HU-naive group (33.3% vs. 10.3%, P=0.009), while 79.4% of HU-group against 56.7% HU-naive had normal body mass index percentile for age and sex, P=0.028. None of the HU-group compared with 13.3% of the HU-naive had severe vitamin D deficiency, P=0.002. The mean 25-OHD of the HU-group was also higher (24.1±1.2 vs. 19.1±9.8 ng/mL, P=0.007). Conclusions: HU possibly ameliorate growth retardation and vitamin D deficiency in children with SCD.
Samuel A. Adegoke, Josefina A.P. Braga, Adekunle D. Adekile, and Maria S. Figueiredo
Ovid Technologies (Wolters Kluwer Health)
Although vitamin D deficiency (VDD) has been linked to anemia among sickle cell disease (SCD), its relationship with hemolysis is unclear. Serum 25-hydroxyvitamin D and biomarkers of hemolysis (hemoglobin [Hb]/hematocrit, reticulocyte percentage, absolute reticulocyte, and lactate dehydrogenase [LDH] levels) in 36 hydroxyurea-naive SCD children were quantified. Correlations were significantly positive with Hb/hematocrit (r=0.40, P=0.017; r=0.45, P=0.006, respectively); inverse with reticulocyte percentage, absolute reticulocyte, and LDH (r=−0.44, P=0.008; r=−0.47, P=0.007; r=−0.45, P=0.007, respectively). In VDD groups, Hb was lower (P=0.014), reticulocyte counts and LDH were higher (P=0.047 and 0.003, respectively). Serum 25-hydroxyvitamin D correlated with biomarkers of hemolysis in SCD and VDD may play a role in SCD pathogenesis.
Samuel A Adegoke, Maria S Figueiredo, Adekunle D Adekile, and Josefina A P Braga
Oxford University Press (OUP)
Background
Comparative studies of patients in different sociogeographic/ecological zones may unravel potential environmental and nutritional factors influencing disease phenotype. In sickle cell disease (SCD), differential access to comprehensive care may influence their growth and nutritional status.
Methods
From June 2015 to February 2016, steady-state nutritional parameters of 109 Brazilian and 95 Nigerian children with SCD attending routine clinic visits at Universidade Federal de São Paulo, Brazil and Obafemi Awolowo University Teaching Hospital, Ile-Ife (Ilesa unit), respectively, were compared.
Results
A relatively high proportion of the children in both centres (23.5%) were wasted [body-mass index (BMI)-for-age z-score<-2). BMI-for-age z-score, height-for-age z-score, upper arm fat area and fat percentage were lower in the Nigerian cohorts. More Nigerians, 29.5% (28/95) against 18.3% (20/109) were wasted, and had short stature, [12.6% (12/95) vs. 3.7% (4/109)] than Brazilians. A higher proportion of Brazilian patients were overweight or obese (9.2 vs. 4.3%), and taller for age (15.6 vs. 8.4%). None of the Nigerian patients had severe vitamin D deficiency, only 12.6% (12/95) had suboptimal vitamin D and 1.1% (1/95) had low serum zinc levels, unlike 79.8% (87/109) of the Brazilian patients with suboptimal vitamin D and 10.1% (11/109) with low zinc.
Conclusion
Undernutrition is still prevalent among the two cohorts. Nigerian patients were thinner and had reduced linear growth for age. This observation justifies the continued need for specialized nutritional care for children with SCD. In addition to hydroxyurea therapy, research is needed to determine appropriate nutritional intervention and exercise regimens for these children.
Ana C. Cabañas‐Pedro, Suely Roizenblatt, Altay A. L. de Souza, Sérgio Tufik, and Maria S. Figueiredo
Wiley
SummaryHigh frequency of periodic limb movements in sleep (PLMS) has been described among children with sickle cell disease (SCD), but there is little information about PLMS among adults with SCD. We aim to determine the frequency of PLMS among adults with SCD and to identify possible associations with iron status and haemolytic parameters. We analysed polysomnography on 99 adults: 74 with sickle cell anaemia (HbSS), 19 with HbSC (double heterozygosis HbS and HbC) and 6 with HbS‐beta thalassaemia. Laboratory data were collected close to the time of the polysomnography examination. The prevalence of PLMS > 5/h was 70% and of PLMS > 15/h 36%, in the total group of patients. No differences were observed regarding gender, use of hydroxyurea and iron parameters. Logistic regression showed an association between PLMS > 15/h and hemolytic parameters: absolute reticulocyte count (p = 0.03) and unconjugated bilirubin (p = 0.01). Our data suggest that PLMS may be associated with manifestations of greater severity in SCD.
Clovis Paniz, Juliano Felix Bertinato, Maylla Rodrigues Lucena, Eduardo De Carli, Patrícia Mendonça da Silva Amorim, Guilherme Wataru Gomes, Cecília Zanin Palchetti, Maria Stella Figueiredo, Christine M Pfeiffer, Zia Fazili,et al.
Elsevier BV
Background: The effects of high-dose folic acid (FA) supplementation in healthy individuals on blood folate concentrations and immune response are unknown.Objective: The aim of the study was to evaluate the effects of daily consumption of a tablet containing 5 mg FA on serum folate; number and cytotoxicity of natural killer (NK) cells; mRNA expression of dihydrofolate reductase (DHFR), methylenetetrahydrofolate reductase (MTHFR), interferon γ (IFNG), tumor necrosis factor α (TNFA), and interleukin 8 (IL8) genes; and concentrations of serum inflammatory markers.Methods: This prospective clinical trial was conducted in 30 healthy Brazilian adults (15 women), aged 27.7 y (95% CI: 26.4, 29.1 y), with a body mass index (in kg/m2) of 23.1 (95% CI: 22.0, 24.3). Blood was collected at baseline and after 45 and 90 d of the intervention. Serum folate concentrations were measured by microbiological assay and HPLC-tandem mass spectrometry [folate forms, including unmetabolized folic acid (UMFA)]. We used real-time polymerase chain reaction to assess mononuclear leukocyte mRNA expression and flow cytometry to measure the number and cytotoxicity of NK cells.Results: Serum folate concentrations increased by ∼5-fold after the intervention (P < 0.001), and UMFA concentrations increased by 11.9- and 5.9-fold at 45 and 90 d, respectively, when compared with baseline (P < 0.001). UMFA concentrations increased (>1.12 nmol/L) in 29 (96.6%) participants at day 45 and in 26 (86.7%) participants at day 90. We observed significant reductions in the number (P < 0.001) and cytotoxicity (P = 0.003) of NK cells after 45 and 90 d. Compared with baseline, DHFR mRNA expression was higher at 90 d (P = 0.006) and IL8 and TNFA mRNA expressions were higher at 45 and 90 d (P = 0.001 for both).Conclusion: This noncontrolled intervention showed that healthy adults responded to a high-dose FA supplement with increased UMFA concentrations, changes in cytokine mRNA expression, and reduced number and cytotoxicity of NK cells. This trial was registered at www.ensaiosclinicos.gov.br as RBR-2pr7zp.