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Resumo Introdução O câncer tem impacto na vida das crianças e seus familiares. As Histórias em Quadrinhos podem ser uma estratégia de fortalecer o vínculo e a comunicação entre profissional/paciente/família. Objetivo Desenvolver e validar um material instrucional/educativo, no formato de Histórias em Quadrinhos, voltada para crianças hospitalizadas com leucemia linfóide aguda. Metodologia Estudo metodológico desenvolvido em nove etapas: elaboração do projeto de pesquisa; definição e seleção do conteúdo; adaptação da linguagem; inclusão de ilustrações; construção de um material piloto; validação do material; layout; impressão final e disponibilização. A validação ocorreu com 10 especialistas entre março e maio de 2022, utilizando-se o Instrumento de Validação de Conteúdo Educativo em Saúde. Resultados Foram elaboradas 5 Histórias em Quadrinhos, com 6 personagens principais, sendo necessárias 63 horas de trabalho. Elas foram divididas por temáticas (distúrbios gastrointestinais; cistite hemorrágica; problemas relacionados a autoestima e autoimagem; risco de infecção e dor óssea) que obtiveram Índice de Validade de Conteúdo global satisfatório entre 0,78 e 0,87. Conclusões e implicações para a prática As histórias em quadrinhos podem ser utilizadas como fonte atrativa e confiável de informações sobre a doença, servindo como apoio às informações durante a internação hospitalar e o preparo para alta.
Resumen Introducción El cáncer tiene un impacto en la vida de los niños y sus familias. Los cómics pueden ser una estrategia para fortalecer el vínculo y la comunicación entre profesional/paciente/familia. Objetivo Desarrollar y validar un material didáctico/educativo, en formato de Historietas, dirigido a niños hospitalizados con leucemia linfocítica aguda. Metodología Estudio metodológico desarrollado en nueve etapas: elaboración del proyecto de investigación; definición y selección de contenidos; adaptación lingüística; inclusión de ilustraciones; construcción de un material piloto; validación del material; disposición; impresión final y disponibilidad. La validación se realizó con 10 especialistas entre marzo y mayo de 2022, utilizando el Instrumento de Validación de Contenido de Educación en Salud. Resultados Se crearon 5 Comics, con 6 personajes principales, requiriendo 63 horas de trabajo. Fueron divididos por temas (trastornos gastrointestinales; cistitis hemorrágica; problemas relacionados con la autoestima y la autoimagen; riesgo de infección y dolor óseo) que obtuvieron un Índice de Validez de Contenido global satisfactorio entre 0,78 y 0,87. Conclusiones e implicaciones para la práctica Los cómics pueden ser utilizados como una fuente atractiva y confiable de información sobre la enfermedad, apoyando información durante la hospitalización y preparación para el alta.
Abstract Introduction Cancer has an impact on the lives of children and their families. Comics can be a strategy to strengthen the bond and communication between professional/patient/family. Objective To develop and validate an instructional/educational material, in the format of Comics, aimed at children hospitalized with acute lymphocytic leukemia. Methodology Methodological study developed in nine stages: preparation of the research project; content definition and selection; language adaptation; inclusion of illustrations; construction of a pilot material; validation of the material; layout; final printing and availability. Validation took place with 10 specialists between March and May 2022, using the Health Education Content Validation Instrument. Results 5 Comics were created, with 6 main characters, requiring 63 hours of work. They were divided by themes (gastrointestinal disorders; hemorrhagic cystitis; problems related to self-esteem and self-image; risk of infection and bone pain) that obtained a satisfactory global Content Validity Index between 0.78 and 0.87. Conclusions and implications for practice Comics can be used as an attractive and reliable source of information about the disease, supporting information during hospitalization and preparation for discharge.
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Humanos , Masculino , Feminino , Criança , Adulto , Pessoa de Meia-Idade , Saúde da Criança , Leucemia-Linfoma Linfoblástico de Células Precursoras , Romances Gráficos como Assunto , Jogos e Brinquedos , NáuseaRESUMO
Acute leukemias (AL) are aggressive neoplasms with high mortality rates. Metabolomics and oxidative status have emerged as important tools to identify new biomarkers with clinical utility. To identify the metabolic differences between healthy individuals (HI) and patients with AL, a multiplatform untargeted metabolomic and lipidomic approach was conducted using liquid and gas chromatography coupled with quadrupole-time-of-flight mass spectrometry (LC-QTOF-MS or GC-QTOF-MS). Additionally, the total antioxidant capacity (TAC) was measured. A total of 20 peripheral blood plasma samples were obtained from patients with AL and 18 samples from HI. Our analysis revealed 135 differentially altered metabolites in the patients belonging to 12 chemical classes; likewise, the metabolic pathways of glycerolipids and sphingolipids were the most affected in the patients. A decrease in the TAC of the patients with respect to the HI was evident. This study conducted with a cohort of Colombian patients is consistent with observations from other research studies that suggest dysregulation of lipid compounds. Furthermore, metabolic differences between patients and HI appear to be independent of lifestyle, race, or geographic location, providing valuable information for future advancements in understanding the disease and developing more global therapies.
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Introduction: The clarification of etiopathology, the improvement of chemotherapy regimens and their risk stratifications, and the improvement in treatment support have increased the survival of children and adolescents affected by Acute Lymphoblastic Leukemia (ALL) past few years. This study aimed to estimate overall survival (OS) and event-free survival (EFS) in an onco-hematology treatment center in Brazil, reports the main clinical-laboratory characteristics of patients at diagnosis, verify the frequency of treatment-related adverse effects and the main causes of death. Material and methods: Retrospective analysis involving patients diagnosed with ALL, treated with the protocol of the Brazilian Group for Treatment of Leukemias in Childhood (GBTLI), between 2010 and 2020 was carried out; the outcomes (relapse, deaths, development of new neoplasms) were analyzed SPSS® software was used for the statistical analyses, and the p-value was considered significant when less than 0.05 for all analyses. Results: 109 patients were included in the study; the median age was 5 years, with a slight predominance of males. Sixty-six patients were classified as high-risk (HR) group and 43 patients were classified as low-risk (LR) group. After 5 years of diagnosis, the OS was 71.5%, and the EFS was 65%. No statistical difference was found between the HR and LR groups for OS and EFS, while leukocyte counts were statistically associated with the outcome of death (p = 0.028). Among the patients, 28 (25.6%) died due to infection accounting 46.4% of death causes. Among the 34 patients with unfavorable outcomes (death and/or relapse), 32 had no research for the minimal residual disease at the end of remission induction, and 25 were not investigated for the presence of chromosomal abnormalities. The most reported complications and treatment-related adverse effects were increased liver transaminases (85.9%), airway infection (79.4%), oral mucositis (67.2%), febrile neutropenia (64.4%), and diarrhea (36.4%). Conclusions: The rates of OS and EFS obtained in this cohort are similar to those obtained in the few previous similar studies in Brazil and lower than those carried out in developed countries. The unavailability of prognostic tests may have hindered risk stratification and influenced the results obtained.
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BACKGROUND: The epidemiology of invasive aspergillosis (IA) in patients with acute lymphoid leukemia (ALL) has not been well characterized. OBJECTIVES: To identify potential peculiarities in the natural history, treatment response and outcome of IA diagnosed in patients with ALL and AML. METHODS: This is a retrospective cohort study conducted in seven tertiary-care hospitals between 2009 and 2017 of all consecutive episodes of IA occurring in adult patients with acute leukemia. Demographic characteristics, underlying disease and recent treatment, antifungal prophylaxis, neutropenia, receipt of corticosteroids, clinical and radiological findings, mycological results, antifungal therapy, and 6-week and 12-week survival were recorded. RESULTS: We identified 77 cases of IA in 54 patients with AML and 23 patients with ALL. The majority of patients developed IA in the context of induction chemotherapy for newly diagnosed (48.0%) or relapsed (41.6%) leukemia, with no differences between ALL and AML. Lung involvement was more frequent in AML (96.3% vs. 82.6%, p = 0.06) and rhinosinusitis was more common in ALL (43.5% vs. 24.1%, p = 0.09). Galactomannan was the microbiologic documentation of IA in 76.6%, with similar patterns of positivity in AML and ALL. The 6-week survival of IA in patients with AML and ALL was 63.0% and 56.5%, respectively (p = 0.60). CONCLUSIONS: The epidemiology, clinical presentation, diagnosis and outcome of IA in ALL patients are similar to patients with AML.
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Aspergilose , Infecções Fúngicas Invasivas , Leucemia Mieloide Aguda , Leucemia-Linfoma Linfoblástico de Células Precursoras , Adulto , Humanos , Antifúngicos/uso terapêutico , Estudos Retrospectivos , Aspergilose/diagnóstico , Aspergilose/tratamento farmacológico , Aspergilose/epidemiologia , Leucemia Mieloide Aguda/complicações , Leucemia Mieloide Aguda/tratamento farmacológico , Infecções Fúngicas Invasivas/tratamento farmacológico , Infecções Fúngicas Invasivas/epidemiologiaRESUMO
L-asparaginase (ASNase) is an efficient inhibitor of tumor development, used in chemotherapy sessions against acute lymphoblastic leukemia (ALL) tumor cells; its use results in 80% complete remission of the disease in treated patients. Saccharomyces cerevisiae's L-asparaginase II (ScASNaseII) has a high potential to substitute bacteria ASNase in patients that developed hypersensitivity, but the endogenous production of it results in hypermannosylated immunogenic enzyme. Here we describe the genetic process to acquire the ScASNaseII expressed in the extracellular medium. Our strategy involved a fusion of mature sequence of protein codified by ASP3 (amino acids 26-362) with the secretion signal sequence of Pichia pastoris acid phosphatase enzyme; in addition, this DNA construction was integrated in P. pastoris Glycoswitch® strain genome, which has the cellular machinery to express and secrete high quantity of enzymes with humanized glycosylation. Our data show that the DNA construction and strain employed can express extracellular asparaginase with specific activity of 218.2 IU mg-1. The resultant enzyme is 40% more stable than commercially available Escherichia coli's ASNase (EcASNaseII) when incubated with human serum. In addition, ScASNaseII presents 50% lower cross-reaction with anti-ASNase antibody produced against EcASNaseII when compared with ASNase from Dickeya chrysanthemi.
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Antineoplásicos , Leucemia-Linfoma Linfoblástico de Células Precursoras , Saccharomyces , Humanos , Asparaginase/metabolismo , Saccharomyces cerevisiae/metabolismo , Saccharomyces/metabolismo , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Antineoplásicos/farmacologiaRESUMO
Introducción: Los pacientes que ingresan a la unidad de cuidados intensivos pediátricos son aquellos con alto riesgo de mortalidad que pueden presentar síndrome de disfunción orgánica múltiple. Los pacientes que padecen leucemia linfoide aguda forman parte de este grupo. Objetivos: Validar la escala pediátrica de evaluación del fallo multiorgánico secuencial (pSOFA) en pacientes cubanos graves con diagnóstico de leucemia linfoide aguda. Métodos: Se realizó un estudio observacional, prospectivo, multicéntrico, en unidades de cuidados intensivos de hospitales cubanos con 92 pacientes y 184 ingresos. Se calcularon las puntuaciones de las escalas de disfunción multiorgánica secuencial, riesgo de mortalidad e índice de mortalidad pediátrica, y se evaluó la presencia de disfunción orgánica en las primeras 24 h y a las 48 h. Resultados: La puntuación pSOFA fue mayor en los no supervivientes (p < 0,001) y la mortalidad se incrementó de modo progresivo en los subgrupos con las puntuaciones pSOFA más altas. El análisis de las curvas de las características operativas del receptor (ROC) mostró que el área bajo la curva (AUC) para la predicción de la mortalidad con la puntuación pSOFA fue de 0,89, comparado con 0,84 y 0,79 con las escalas PRISM-3 y PIM-2, respectivamente. Conclusiones: La escala pSOFA mostró ser útil para establecer los criterios disfunción orgánica y su especificidad en el riesgo de mortalidad en los pacientes pediátricos cubanos críticos con diagnóstico de leucemia linfoide aguda(AU)
Introduction: Patients admitted to the pediatric intensive care unit (PICU) are those with a high risk of mortality who may present multiple organ dysfunction syndrome. Patients with acute lymphoid leukemia are part of this group. Objectives: To validate the pediatric sequential multi-organ failure assessment scale (pSOFA) in severe Cuban patients diagnosed with acute lymphoid leukemia. Methods: An observational, prospective, multicenter study was carried out in intensive care units of Cuban hospitals with 92 patients and 184 admissions. The scores of the sequential multiple organ dysfunction, mortality risk and pediatric mortality index scales were calculated, and the presence of organ dysfunction was evaluated in the first 24 hours and at 48 hours. Results: The pSOFA score was higher in non-survivors (p <0.001) and mortality progressively increased in the subgroups with the highest pSOFA scores. The analysis of the receiver operating characteristics (ROC) curves showed that the area under the curve (AUC) for the prediction of mortality with the pSOFA score was 0.89, compared to 0.84 and 0.79 with the PRISM-3 and PIM-2 scales, respectively. Conclusions: The pSOFA scale proved useful to establish the criteria for organ dysfunction and its specificity in the risk of mortality in critical Cuban pediatric patients diagnosed with acute lymphoid leukemia(AU)
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Humanos , Recém-Nascido , Lactente , Pré-Escolar , Criança , Unidades de Terapia Intensiva Pediátrica , Sensibilidade e Especificidade , Leucemia-Linfoma Linfoblástico de Células Precursoras , Insuficiência de Múltiplos Órgãos , Pesos e Medidas , Estudos Prospectivos , Curva ROC , Estudo ObservacionalRESUMO
In Brazil, Acute lymphoid leukemia (ALL) is the leading cause of cancer deaths in children and adolescents. Treatment toxicity is one of the reasons for stopping chemotherapy. Amerindian genomic ancestry is an important factor for this event due to fluctuations in frequencies of genetic variants, as in the NUDT15 and SLC22A1 genes, which make up the pharmacokinetic and pharmacodynamic pathways of chemotherapy. This study aimed to investigate possible associations between NUDT15 (rs1272632214) and SLC22A1 (rs202220802) gene polymorphism and genomic ancestry as a risk of treatment toxicities in patients with childhood ALL in the Amazon region of Brazil. The studied population consisted of 51 patients with a recent diagnosis of ALL when experiencing induction therapy relative to the BFM 2009 protocol. Our results evidenced a significant association of risk of severe infectious toxicity for the variant of the SLC22A1 gene (OR: 3.18, p = 0.031). Genetic ancestry analyses demonstrated that patients who had a high contribution of African ancestry had a significant protective effect for the development of toxicity (OR: 0.174; p = 0.010), possibly due to risk effects of the Amerindian contribution. Our results indicate that mixed populations with a high degree of African ancestry have a lower risk of developing general toxicity during induction therapy for ALL. In addition, individuals with the SLC22A1 variant have a higher risk of developing severe infectious toxicity while undergoing the same therapy.
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Transportador 1 de Cátions Orgânicos , Leucemia-Linfoma Linfoblástico de Células Precursoras , Adolescente , População Negra , Criança , Humanos , Transportador 1 de Cátions Orgânicos/genética , Polimorfismo Genético , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Leucemia-Linfoma Linfoblástico de Células Precursoras/genética , Pirofosfatases/genéticaRESUMO
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 Hematology Oncology and Cellular Therapy to make recommendations on the current scenario of the use of CAR-T cells in patients with ALL.
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Introducción: El pronóstico de las enfermedades hematológicas malignas ha experimentado un importante avance en las últimas décadas, sobre todo por las nuevas combinaciones de quimioterapia. Estos hechos han propiciado que muchos de estos pacientes, en algún momento de su enfermedad, sean tratados en unidades de cuidados intensivos, lo que no era frecuente hace dos décadas. Objetivo: Describir el desarrollo de la disfunción múltiple de órganos en pacientes pediátricos con leucemia linfoide aguda en terapia intensiva en el Instituto de Hematología e Inmunología. Métodos: Se realizó un estudio clínico, observacional, transversal en el que se incluyeron los pacientes pediátricos con leucemia linfoblástica aguda y disfunción múltiple de órganos, atendidos en el servicio de terapia intensiva en el periodo 2018 a 2020. Se analizaron las variables: sociodemográficas, estado nutricional, diagnóstico al ingreso, puntaje del score pSOFA, conducta fármaco-terapéutica. Resultados: El grupo de edad más afectado fue el de 1 a 4 años, en su mayoría normopesos, con complicaciones de choque séptico, distrés respiratorio, y con 33 por ciento de mortalidad mayor en aquellos pacientes con score pSOFA con más de 10 puntos. La conducta terapéutica más utilizada fue la administración de oxígeno, fluidoterapia y antibióticos de tercera y cuarta generación en la primera hora de ingreso al servicio. Conclusiones: Si el puntaje del score pSOFA es mayor de 10 puntos existe mayor riesgo de muerte y mortalidad pediátrica (90 por ciento )(AU)
Introduction: The prognosis of hematological malignancies has undergone an important advance in the last decades, mainly due to the new chemotherapy combinations. These facts have led many of these patients to be treated in intensive care units at some point during their illness. Objective: To describe the development of multiple organ dysfunction in pediatric patients with acute lymphoid leukemia in intensive care at the Institute of Hematology and Immunology. Methods: A clinical, observational, cross-sectional study was carried out that included pediatric patients with acute lymphoblastic leukemia and multiple organ dysfunction, treated in the intensive care service in the period from 2018 to 2020. The variables were analyzed: sociodemographic, nutritional status, diagnosis on admission, pSOFA score, drug-therapeutic behavior. Results: The most affected age group was 1 to 4 years old, mostly normal weight, with complications of septic shock, respiratory distress, and 33 percent higher mortality in those patients with a pSOFA score with more than 10 points. The most used therapeutic approach was the administration of oxygen, fluid therapy and third and fourth generation antibiotics in the first hour of admission to the service. Conclusions: If the pSOFA score is greater than 10 points, there is a greater risk of death and pediatric mortality (90 percent)(AU).
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Humanos , Lactente , Pré-Escolar , Leucemia-Linfoma Linfoblástico de Células Precursoras , Escores de Disfunção Orgânica , Unidades de Terapia Intensiva , Cooperação Internacional , Antibacterianos , Estudos TransversaisRESUMO
Introducción: Durante el tratamiento de inducción de la leucemia linfoide aguda en niños no siempre se identifican las reacciones adversas a medicamentos. Objetivo: Describir los eventos adversos y las reacciones adversas a medicamentos durante el tratamiento de inducción de la leucemia linfoide aguda, en niños tratados en el Instituto de Hematología e Inmunología de Cuba, durante 2012-2017. Método: Estudio observacional, descriptivo, transversal, de series de casos en farmacovigilancia, se utilizó la farmacovigilancia activa. Variables: sexo, edad, grupo pronóstico, semana de tratamiento, tipo de evento adverso, sistema de órgano afectado, severidad e imputabilidad. La información se obtuvo del registro nacional del protocolo ALLIC-BFM 2009 y las historias clínicas. Resultados: Se incluyeron 69 niños, 55,1 por ciento (38 casos) fueron masculinos, 56,5 por ciento (39 niños) tenía entre uno y seis años. El 52,2 por ciento (36 pacientes) pertenecían al grupo pronóstico intermedio. Se registraron 471 eventos adversos. El 50,5 por ciento (238/471) ocurrió en la primera semana de tratamiento. Los más frecuentes: anemia (17,8 por ciento; 84/471), neutropenia (16,1 por ciento; 76/471) y trombocitopenia (15,9 por ciento; 75/471). Los sistemas de órganos más afectados: hemolinfopoyético (57,54 por ciento; 271/471) y gastrointestinal (15,71 por ciento; 74/471). El 93,2 por ciento (439/471) se clasificó en reacciones adversas posibles. Según gravedad el 72,4 por ciento (330/456) fueron moderadas y el 27,4 por ciento (125/456) graves. Conclusiones: Todos los casos presentaron eventos adversos, predominaron las alteraciones hematológicas y los eventos reportados para fármacos incluidos en la quimioterapia. Se identificaron reacciones adversas clasificadas como posibles, con predominio de las moderadas y graves(AU)
Introduction: During the induction treatment of acute lymphoid leukemia in children, adverse drug reactions are not always identified. Aims: Describe the demographic and clinical characteristics of children with acute lymphoid leukemia who receive induction treatment at the Institute of Hematology and Immunology between 2012-2017. Characterize adverse events that occur during induction treatment. Describe adverse drug reactions during induction. Methods: Observational, descriptive, cross-sectional study of case series in pharmacovigilance, used active pharmacovigilance. Variables: sex, age, prognosis group, week of treatment, type of adverse event, organ system affected, severity and imputability. The information was obtained from the national register of the ALLIC-BFM 2009 protocol and the medical records. Results: 69 children were included, 55.1 percent belonged to the male sex, 56.5 percent were between one and six years old. 52.2 percent (36 children) belonged to the intermediate prognosis group. 471 events were recorded. 50.5 percent occurred in the first week of treatment. The most frequent: anemia (17.8 percent), neutropenia (16.1 percent) and thrombocytopenia (15.9 percent). The most affected organ systems: hemolinfopoietic (57.5 percent) and gastrointestinal (15.7 percent). According to the severity, 72.4 percent were moderate and 27.4 percent severe. Conclusions: The whole presented adverse events, hematological alterations and reported events for drugs included in chemotherapy predominated. Adverse reactions classified as possible were identified, moderate and severe predominated(AU)