Children and adolescents with non-Hodgkin lymphoma in Brazil: A national survey identifies the challenges encountered and documents the diversity of care

ABSTRACT Introduction: Brazil has many inequities in the healthcare provided nationwide. Therefore, in order to access challenges in treatment, available resources and current practices, to identify barriers in delivering a good quality of care among Brazilian centers treating children and adolescents with Non-Hodgkin Lymphoma (NHL) and to generate a future prospective guideline, a customized online survey was distributed to pediatric hematologists and oncologists across the country. Results: A total of 97 surveys were completed (35% response rate), from 47 cities in all Brazilian regions and 79 units of care, with a median of 1 answer by the center (range 1 - 5). Most respondents work at an institution supported exclusively by public/philanthropic resources (58%), with an average of 5 to 9 new cases/year (49%), and 41% have 4 to 6 oncologists/centers. Additionally, 22% have no easy access to the intensive care unit, 26% have no access to Rasburicase, 28% have no access to Rituximabe as front-line therapy and 41% have unreliable methotrexate monitoring levels. Those differences cannot be explained thoroughly by regional wealth variances, nor by the financing model. Regarding the pathology service, 70% consider having reasonable quality assistance, but the timeframe to deliver diagnosis is satisfactory to 46%. There is no uniform management of care, with the current guideline from the Sociedade Brasileira de Oncologia Pediátrica being adopted by 54 to 59%, depending on the NHL subtype. Conclusion: This study provides insights into the heterogeneity of care among Brazilian centers. Recognizing those diversities will support the design of effective strategies and collaboration nationwide.

Children and adolescents with non-Hodgkin lymphoma in Brazil: A national survey identifies the challenges encountered and documents the diversity of care.

INTRODUCTION: Brazil has many inequities in the healthcare provided nationwide. Therefore, in order to access challenges in treatment, available resources and current practices, to identify barriers in delivering a good quality of care among Brazilian centers treating children and adolescents with Non-Hodgkin Lymphoma (NHL) and to generate a future prospective guideline, a customized online survey was distributed to pediatric hematologists and oncologists across the country. RESULTS: A total of 97 surveys were completed (35% response rate), from 47 cities in all Brazilian regions and 79 units of care, with a median of 1 answer by the center (range 1 - 5). Most respondents work at an institution supported exclusively by public/philanthropic resources (58%), with an average of 5 to 9 new cases/year (49%), and 41% have 4 to 6 oncologists/centers. Additionally, 22% have no easy access to the intensive care unit, 26% have no access to Rasburicase, 28% have no access to Rituximabe as front-line therapy and 41% have unreliable methotrexate monitoring levels. Those differences cannot be explained thoroughly by regional wealth variances, nor by the financing model. Regarding the pathology service, 70% consider having reasonable quality assistance, but the timeframe to deliver diagnosis is satisfactory to 46%. There is no uniform management of care, with the current guideline from the Sociedade Brasileira de Oncologia Pediátrica being adopted by 54 to 59%, depending on the NHL subtype. CONCLUSION: This study provides insights into the heterogeneity of care among Brazilian centers. Recognizing those diversities will support the design of effective strategies and collaboration nationwide.

Pediatric Acute Promyelocytic Leukemia: Epidemiology, Molecular Features, and Importance of GST-Theta 1 in Chemotherapy Response and Outcome.

Previous studies have suggested a variation in the incidence of acute promyelocytic leukemia (APL) among the geographic regions with relatively higher percentages in the Latin American population. We aimed to explore the population burden of pediatric APL, gathering information from the population-based cancer registry (PBCR) and the diagnosis of APL obtained through incident cases from a hospital-based cohort. The homozygous deletion in glutathione S-transferases (GSTs) leads to a loss of enzyme detoxification activity, possibly affecting the treatment response. Mutations in the RAS pathway genes are also considered to be a key component of the disease both in the pathogenesis and in the outcomes. We have assessed mutations in a RAS-MAP kinase pathway (FLT3, PTPN11, and K-/NRAS) and GST variant predisposition risk in the outcome. Out of the 805 children and adolescents with acute myeloid leukemia (AML) who are registered in the PBCR, 35 (4.3%) were APL cases. The age-adjusted incidence rate (AAIR) was 0.03 per 100,000 person-years. One-hundred and sixty-three patients with APL were studied out of 931 AML cases (17.5%) from a hospital-based cohort. Mutations in FLT3, KRAS, and NRAS accounted for 52.1% of the cases. Patients with APL presented a 5-year probability of the overall survival (OS) of 67.3 ± 5.8%. A GST-theta 1 (GSTT1) null genotype conferred adverse prognosis, with an estimated hazard ratio of 2.8, 95% confidence interval (CI) 1.2-6.9. We speculate that the GSTT1 polymorphism is associated with therapeutics and would allow better OS of patients with APL with a GSTT1 null genotype.

Prevalência de anemia em pré-escolares e resposta ao tratamento com suplementação de ferro / Prevalence of anemia among preschoolers and response to iron supplementation

OBJETIVOS: Avaliar a prevalência de anemia entre os pré-escolares da rede pública de Ilhabela (SP) e a resposta ao tratamento instituído. MÉTODOS: Estudo com 667 pré-escolares, entre 2007 e 2008. As crianças foram avaliadas na escola quanto ao peso, estatura e concentração de hemoglobina. As anêmicas foram tratadas com sulfato ferroso por 12 semanas, com uma consulta intermediária e outra ao final. RESULTADOS: A prevalência de anemia foi de 25,6 por cento (170 crianças). A concentração de hemoglobina mais baixa foi de 9,5 g/dL. A maior prevalência de anemia (36 por cento) ocorreu em crianças de 60 a 65 meses de idade. A mediana da concentração de hemoglobina evoluiu de 10,5 g/dL para 11,8 g/dL ao final do tratamento, recuperando 76 por cento das crianças. CONCLUSÃO: A identificação de crianças anêmicas nas escolas, o pronto tratamento da anemia e o acompanhamento durante o tratamento mostrou ser estratégia eficaz no combate a esse importante problema de saúde pública.

OBJECTIVES: To assess the prevalence of anemia among children attending public preschools in Ilhabela, state of São Paulo, Brazil, and their response to a treatment regimen. METHODS: Between 2007 and 2008, the weight, height, and hemoglobin levels of 667 children were measured in the school setting. Anemic children were prescribed a 12-week course of ferrous sulfate, and follow-up assessment visits were scheduled for halfway through this course and the end of treatment. RESULTS: The prevalence of anemia was 25.6 percent (N = 170). The lowest hemoglobin level measured was 9.5 g/dL. Anemia was most prevalent (36 percent) in children between the ages of 60 and 65 months. Median hemoglobin levels rose to 11.8 g/dL from 10.5 g/dL after treatment; 76 percent of children recovered from anemia. CONCLUSION: Screening for anemia in the school setting and prompt therapy, including mid-treatment follow-up, proved to be an effective strategy for facing this major public health issue.

Prevalence of anemia among preschoolers and response to iron supplementation.

OBJECTIVES: To assess the prevalence of anemia among children attending public preschools in Ilhabela, state of São Paulo, Brazil, and their response to a treatment regimen. METHODS: Between 2007 and 2008, the weight, height, and hemoglobin levels of 667 children were measured in the school setting. Anemic children were prescribed a 12-week course of ferrous sulfate, and follow-up assessment visits were scheduled for halfway through this course and the end of treatment. RESULTS: The prevalence of anemia was 25.6% (N = 170). The lowest hemoglobin level measured was 9.5 g/dL. Anemia was most prevalent (36%) in children between the ages of 60 and 65 months. Median hemoglobin levels rose to 11.8 g/dL from 10.5 g/dL after treatment; 76% of children recovered from anemia. CONCLUSION: Screening for anemia in the school setting and prompt therapy, including mid-treatment follow-up, proved to be an effective strategy for facing this major public health issue.