Evolución clínica en neuromielitis óptica comparando el manejo con azatioprina o rituximab / Clinical evolution in neuromyelitis optica when comparing management with azathioprine and rituximab

RESUMEN INTRODUCCIÓN: El espectro de trastornos de neuromielitis óptica (NMOSD) es un grupo de enfermedades desmielinizantes, inflamatorias y autoinmunes, caracterizadas por episodios recurrentes de neuritis óptica y mielitis transversa longitudinal extensa, entre otras manifestaciones clínicas. Su tratamiento crónico se basa en el uso de terapias inmunosupresoras como azatioprina (AZA), micofenolato mofetilo (MFM) o rituximab (RTX). El objetivo del presente estudio es realizar un análisis comparativo de la respuesta al tratamiento con AZA o RTX. MATERIALES Y MÉTODOS: Se realizó un estudio observacional, analítico, retrospectivo, en el cual se incluyeron inicialmente 69 pacientes con diagnóstico confirmado de NMOSD. Tras aplicar los criterios de inclusión y exclusión 59 pacientes fueron incluidos en el análisis final. RESULTADOS: En el grupo de RTX se evidenció una mejoría importante en el estado funcional en comparación con el grupo de AZA, en el que se vio un empeoramiento de este al año de seguimiento. El perfil de seguridad fue similar entre ambos grupos, con una adherencia significativamente superior en el grupo de RTX. DISCUSIÓN: Los hallazgos del presente estudio respecto a las ventajas del uso de RTX sobre AZA se encuentran en concordancia con resultados de estudios previos reportados en la literatura. CONCLUSIONES: Los resultados respaldan el uso de RTX sobre AZA como terapia de mantenimiento para pacientes con NMOSD, al estar asociado principalmente con una mejoría notable en la funcionalidad de los pacientes, al igual que una mayor adherencia al tratamiento.

ABSTRACT INTRODUCTION: Neuromyelitis Optica Spectrum Disorders (NMOSD) is a group of inflammatory, autoimmune, and demyelinating disorders. Its hallmark behavior is characterized by recurrent episodes of optic neuritis and longitudinally extensive transverse myelitis, among other clinical manifestations. Chronic therapy is based primarily in immunosuppressive therapies such as azathioprine (AZA), mycophenolate mofetil (MMF), or rituximab (RTX). The goal of this study is to perform a comparative analysis of response rates to chronic treatment with either AZA or RTX. MATERIALS AND METHODS: A retrospective observational analytic study was designed with an initial cohort of 69 patients with a diagnosis of NMOSD. After application of the inclusion and exclusion criteria a total of 59 patients were finally included in the analysis. RESULTS: The RTX group had an improved functional status when compared to the AZA group; in the latter this feature worsened after a one-year follow-up. There was also a comparable safety profile between the two groups with a significantly greater adherence to RTX regimes. DISCUSSION: The findings of the current study as to the benefits of RTX in comparison to AZA are similar to the results of previous studies. CONCLUSION: These results favor the use of RTX as maintenance treatment of NMOSD, because of its greater benefit mainly in the improvement in functional status of patients, as well as a greater adherence to treatment.

Judicialização no acesso a medicamentos: os números no período de 2019 a 2021 no município de Ponta Grossa, Paraná / Judicialization in access to medicines: the numbers in the period from 2019 to 2021 in the municipality of Ponta Grossa, Paraná

descrever o perfil das ações judiciais quanto ao fornecimento de medicamentos no município de Ponta Grossa, Paraná, entre 2019 a 2021. Metodologia: Utilizou-se o método descritivo-exploratório e retrospectivo, de abordagem quantitativa. Foram analisados 89 processos, sendo grande parte dos autores do sexo feminino (57,3%), com predomínio de assistência jurídica pública por meio do Ministério Público (69,7%) e com prescrições do serviço público (96,6%). Resultados: Observou-se que 93,3% das solicitações não constavam na Relação Nacional de Medicamentos Essenciais. Ademais, verificou-se maior demanda do medicamento Avastin (31,5). As doenças que mais acometeram a parte autora foram retinopatia diabética (42,7%; n=38) e doença pulmonar obstrutiva crônica (24,7%). Destaca-se que no período da coleta de dados, 79,8% dos processos encontravam-se finalizados. Os valores gastos com a judicialização de medicamentos variaram de R$ 407,94 a R$ 47.220,00. Considerações: Haja vista o número não exorbitante de ações judiciais, é possível que existam estratégias municipais efetivas, ou a ausência de Defensoria Pública no atendimento as demandas de saúde, pode ter tornado fator inibidor de acesso à justiça. Outro ponto quanto a delimitação no acesso, são os requisitos cumulativos firmados na tese pelo STF, quanto a concessão de medicamentos não previstos nas listas de dispensação do SUS. Conclui-se que há necessidade de aprimorar o diálogo entre o judiciário e o setor de saúde por meio dos seus gestores, bem como a realização de mais estudos para subsidiar um mapeamento, planejamento, descrição de gastos com a judicialização, aquisição de medicamentos e incorporação de novas tecnologias

Objective: to describe the profile of lawsuits regarding the supply of medicines in the city of Ponta Grossa, Paraná, between 2019 and 2021. Methods: A descriptive-exploratory and retrospective method was used, with a quantitative approach. A total of 89 processes were analyzed, most of which were female authors (57.3%), with a predominance of public legal assistance through the Public Prosecutor's Office (69.7%) and with prescriptions from the public service (96.6%). Results: It was observed that 93.3% of requests were not included in the National List of Essential Medicines. Furthermore, there was a greater demand for the drug Avastin (31.5). The diseases that most affected the author were diabetic retinopathy (42.7%; n=38) and chronic obstructive pulmonary disease (24.7%). It is noteworthy that during the data collection period, 79.8% of the processes were concluded. The amounts spent on the legalization of medicines ranged from R$ 407.94 to R$ 47,220.00. Considerations: Given the not exorbitant number of lawsuits, it is possible that there are effective municipal strategies, or the absence of a Public Defender's Office in meeting health demands, which may have become an inhibiting factor in access to justice. Another point regarding the delimitation of access, are the cumulative requirements signed in the thesis by the STF, regarding the concession of medicines not foreseen in the SUS dispensing lists. It is concluded that there is a need to improve the dialogue between the judiciary and the health sector through its managers, as well as to carry out more studies to support a mapping, planning, description of expenses with the judicialization, acquisition of medicines and incorporation of new technologies

Belimumabe comparado à terapia padrão do Sistema Único de Saúde no tratamento da nefrite lúpica nos estágios III, IV e V: revisão rápida / Belimumab compared to standard therapy of the Brazilian Public Health System in the treatment of lupus nephritis in stage III, IV, and V: rapid review

Belimumabe, rituximabe, terapia imunossupressora. Indicação: Nefrite lúpica nos estágios III, IV, V, refratária à terapia imunossupressora. Pergunta: Belimumabe é eficaz (remissão da nefrite, normalização da perda da função renal, qualidade de vida) e seguro (descontinuação devido a eventos adversos totais e eventos adversos graves) para o tratamento de pacientes com nefrite lúpica refratária nos estágios III, IV, V em comparação aos medicamentos disponíveis no Sistema Único de Saúde? Objetivo: Avaliar a segurança e eficácia do belimumabe em comparação com os medicamentos disponíveis no Sistema Único de Saúde em pacientes adultos com nefrite lúpica. Métodos: Revisão rápida de revisões sistemáticas. Levantamento bibliográfico foi realizado nas bases de dados PUBMED, EMBASE, SCOPUS, BVS, EPISTEMONIKOS, Cochrane Library e em registros de revisões sistemáticas e ensaios clínicos. Seguiu estratégias de buscas predefinidas. Foi feita avaliação da qualidade metodológica dos estudos incluídos através da ferramenta AMSTAR-2 (Assessing the Methodological Quality of Systematic Reviews Version 2). Resultados: Foram selecionadas duas revisões sistemáticas que atendiam aos critérios de elegibilidade, mas nenhum ensaio clínico foi escolhido, pois não atendiam aos critérios de inclusão. Conclusão: a terapia combinada de belimumabe, ou de rituximabe, com tratamento imunossupressor padrão é mais eficaz que o tratamento padrão para alcançar remissão clínica da nefrite lúpica. A terapia combinada é tão segura quanto o tratamento padrão. Belimumabe e rituximabe tem eficácia similar entre si

Belimumab, rituximab, and immunosuppressive therapy. Indication: Refractory lupus nephritis to immunosuppressive therapy in stages III, IV, V. Question: Is belimumab effective (for remission of nephritis, normalization of loss of renal function, quality of life) and safe (for discontinuation due to total adverse events and serious adverse events) in the treatment of patients with refractory lupus nephritis in stages III, IV, V compared to the drugs available in the Brazilian Public Health System? Objective: To evaluate the safety and efficacy of belimumab compared to drugs available in the Brazilian Public Health System in adult patients with lupus nephritis. Methods: Rapid review of systematic reviews. A bibliographic search was done in the PUBMED, EMBASE, SCOPUS, BVS, EPISTEMONIKOS, Cochrane Library databases and in records of systematic reviews and clinical trials. It has followed predefined search strategies. The methodological quality of the included studies was evaluated using the AMSTAR-2 tool (Assessing the Methodological Quality of Systematic Reviews Version 2). Results: Two systematic reviews were selected, which met the eligibility criteria, but no clinical trials were chosen, as they did not meet the inclusion criteria. Conclusion: Combination therapy of belimumab or rituximab with standard immunosuppressive treatment is more effective than standard treatment in achieving clinical remission of lupus nephritis. Combination therapy is as safe as standard treatment. Belimumab and rituximab have similar efficacy to each other

Impacto orçamentário da incorporação do rituximabe para o tratamento de leucemia linfocítica crônica no SUS / Budget impact of the incorporation of rituximab for the treatment of chronic lymphocytic leukemia in Brazil

Objetivo: O presente estudo objetiva desenvolver um modelo de análise de impacto orçamentário (AIO) relacionada à incorporação do rituximabe no tratamento de primeira linha da leucemia linfocítica crônica (LLC) no Sistema Único de Saúde (SUS). Métodos: A elaboração da AIO foi realizada de acordo com as recomendações metodológicas das diretrizes brasileiras, considerando a perspectiva do SUS, horizonte temporal de cinco anos, população a ser tratada, diferentes cenários de market share do rituximabe e custos diretos envolvidos no tratamento atual e no tratamento proposto, e também foi executada uma análise de sensibilidade para avaliar possíveis incertezas futuras. Resultados: A cada ano e ao final do horizonte temporal de cinco anos, a incorporação do rituximabe promoverá aumento dos custos, quando comparado com o valor de ressarcimento do SUS para o tratamento de primeira linha da LLC. No cenário de maior participação de mercado do rituximabe, os custos totais foram menores em relação ao cenário de menor market share. Dado que a estimativa da AIO é para gastos futuros, incertezas relacionadas como a possível elevação do custo do medicamento foi o fator que promoveu o cenário de maiores gastos. Conclusões: A projeção de custos estimados pela AIO demonstrou menores gastos financeiros no cenário de maior difusão do medicamento, o que pode ter correlação com o atraso da progressão da doença ao utilizar o rituximabe, e consequentemente menos pacientes irão requerer segunda linha de tratamento, que tem custo mais elevado.

Objective: This study aims to develop a budget impact analysis (BIA) model related to the incorporation of rituximab in the first-line treatment of chronic lymphocytic leukemia (CLL) in the Unified Health System (SUS). Methods: The preparation of the BIA was carried out in accordance with the methodological recommendations of the Brazilian guidelines, considering the perspective of the SUS, a time horizon of five years, population to be treated, different market share scenarios for rituximab and direct costs involved in the current treatment and treatment proposed, a sensitivity analysis was also performed to assess possible future uncertainties. Results: Each year and at the end of the five-year time horizon, the incorporation of rituximab will increase costs, when compared to the SUS reimbursement value for the first-line treatment of CLL. In the scenario of higher market share for rituximab, total costs were lower compared to the scenario of lower market share. Given that the BIA estimate is for future expenses, uncertainties related to the possible increase in the cost of the drug were the factor that promoted the scenario of higher expenses. Conclusions: The projection of costs estimated by the BIA showed lower financial expenses in the scenario of greater diffusion of the drug, which may be correlated with the delay in the progression of the disease when using rituximab and, consequently, fewer patients will require second-line treatment, which has a higher cost.

Rituximab: alternativa eficaz para la querotopatía ulcerativa periférica / Rituximab: effective alternative for peripheral ulcerative kerotopathy

El desarrollo de los fármacos biológicos ha permitido controlar de manera óptima las manifestaciones oftalmológicas de las enfermedades autoinmunes, el conocimiento de nuevas vías patogénicas y de nuevas dianas terapéuticas lo que está suponiendo una revolución en el tratamiento médico de numerosas entidades y donde la oftalmología no es una excepción. Se presenta el caso clínico de una paciente con artritis reumatoide con mal control y afectación multisistémica que presentó una queratopatía ulcerativa periférica de mal control que respondió con tratamiento biológico diferente al tradicionalmente usado: el rituximab(AU)

The development of biological drugs has allowed optimal control of the ophthalmologic manifestations of autoimmune diseases, the knowledge of new pathogenic pathways and new therapeutic targets, which is leading to a revolution in the medical treatment in numerous entities and where ophthalmology is no exception. We present the clinical case of a patient with poorly controlled rheumatoid arthritis and multisystemic condition. The patient presented a poorly controlled peripheral ulcerative keratopathy that responded to a different biological treatment than the one traditionally used: rituximab(AU)

Linfomas no Hodgkin indolentes y terapia anti-CD20: una revisión de la literatura / Indolent non-Hodgkin's lymphomas and anti-CD20 therapy: a literature review

Introducción: Los linfomas no Hodgkin indolentes se destacan por el reto que suponen desde el punto de vista terapéutico. La introducción de la terapia con rituximab, un anticuerpo monoclonal que se une al antígeno CD20 de la membrana de los linfocitos B, revolucionó los tratamientos hasta ese momento y abrió el camino para el desarrollo de otros anticuerpos monoclonales anti-CD20. Objetivo: Describir las características generales de los linfomas no Hodgkin indolentes y de los anticuerpos monoclonales anti-CD20, así como el rol de la terapia anti-CD20 en dichas enfermedades. Métodos: Se realizó una revisión de la literatura publicada en los últimos 20 años, disponible en los repositorios: Scielo, Scopus, Pubmed/Medline, ScienceDirect y Mediagraphic. Se emplearon para elaborar este manuscrito 35 documentos, de ellos 80 por ciento correspondieron a los últimos 5 años. Conclusiones: La sólida evidencia científica, acumulada durante las últimas dos décadas, respalda el uso clínico de los anticuerpos monoclonales anti-CD20 en el tratamiento de los linfomas no Hodgkin indolentes. El uso efectivo de estos fármacos como agentes únicos o combinados con quimioterapia demuestran su versatilidad terapéutica(AU)

Introduction: Indolent non-Hodgkin's lymphomas are notable for the challenge they pose from a therapeutic point of view. The introduction of rituximab, a monoclonal antibody that binds to the CD20 antigen of the B-lymphocyte membrane, revolutionized treatments up to that time and opened the way for the development of other anti-CD20 monoclonal antibodies. Objective: To describe the general characteristics of indolent non-Hodgkin's lymphomas and anti-CD20 monoclonal antibodies, as well as the role of anti-CD20 therapy in these diseases. Methods: A review of the literature published in the last 20 years, available in the repositories: Scielo, Scopus, Pubmed/Medline, Science Direct and Mediagraphic, was performed. Thirty-five papers were used to prepare this manuscript, 80 percent of which corresponded to the last 5 years. Conclusions: Strong scientific evidence, accumulated over the last two decades, supports the clinical use of anti-CD20 monoclonal antibodies in the treatment of indolent non-Hodgkin's lymphomas. The effective use of these drugs as single agents or in combination with chemotherapy demonstrates their therapeutic versatility(AU)

Enfermedad relacionada a IgG4. Serie clínica de pacientes chilenos / IGG4-related disease. Report of 52 patients

BACKGROUND: IgG4-related disease (IgG4 RD) is an immune-mediated fibro-inflammatory disorder, with tissue infiltration of IgG4+ plasma cells. It causes pseudotumors, tumors, and a wide spectrum of clinical manifestations. AIM: To report the clinical, laboratory, histopathological and treatment characteristics of a group of Chilean patients with IgG4 RD. MATERIAL AND METHODS: Review of medical records of 52 patients aged 18 to 76 years with IgG4 RD seen at six medical centers. RESULTS: Elevated IgG4 serum levels (> 135 mg/dl) were found in 18 of 44 (41%) patients. There was histological confirmation of the disease in 46 patients. The most common sites of involvement were lungs, eyes and kidneys. Eighteen (35%) patients had only one organ involved, 34 (65%) patients had two organs and 13 (25%) patients had three or more organs. The involvement of two organs was significantly more common in men (p < 0.05). In patients with only one organ involvement, the most frequent location was orbital and meningeal. All patients with kidney or lung disease had multiorgan involvement. All patients received corticosteroid therapy, 67% synthetic immunosuppressants, and 16% rituximab. CONCLUSIONS: ER-IgG4 can affect any tissue. Multiorgan involvement was more common in this series, with preference for lungs, eyes and kidneys. An excellent response to steroids is characteristic of the disease, but with a high relapse rate that requires additional immunosuppression.

Boas Práticas na Administração do Rituximab: Revisão Integrativa da Literatura / Good practices in Rituximab Administration: Integrative Literature Review / Buenas Prácticas en la Administración de Rituximab: Revisión Integradora de la Literatura

Introdução: O rituximab é um anticorpo monoclonal quimérico camundongo/humano, amplamente utilizado no cenário terapêutico de vários diagnósticos. Por apresentar diferentes protocolos de administração, manejo e efeitos adversos, seu uso requer atenção da equipe de saúde. Objetivo: Descrever os protocolos infusionais do rituximab na primeira infusão, nas subsequentes e na dessensibilização, e caracterizar a sua segurança. Método: Revisão integrativa da literatura. A busca pelos periódicos foi realizada nas bases de dados e bibliotecas eletrônicas: LILACS, PubMed, MEDLINE, SciELO e BDEnf. Resultados: O cruzamento dos descritores proporcionou a identificação de 413 artigos. Destes, 113 foram lidos na íntegra e, ao final, 16 artigos foram aplicáveis ao desenho do estudo. Os artigos foram publicados entre os anos de 2016 e 2020, com predomínio da língua inglesa (87,5%). Quanto às principais formas de administração do medicamento, nove estudos abordaram a infusão por via intravenosa (em variadas modalidades de tempo) e sete por via subcutânea. Conclusão: De acordo com a literatura científica, todas as modalidades de infusão intravenosa e subcutânea demostram ser seguras e eficazes se os protocolos forem adequadamente indicados e corretamente aplicados

Introduction: Rituximab is a mouse/human chimeric monoclonal antibody, widely used in the therapeutic setting of various diagnoses, due to its different administration, management and adverse effects protocols; its use requires attention by the healthcare team. Objective: Describe the infusion protocols for rituximab in the first, subsequent and desensitization infusions, and characterize their safety. Method: Integrative literature review. The search was performed in databases and electronic libraries: LILACS, PubMed, MEDLINE, SciELO and BDEnf. Results: In all, 413 articles were eligible after crossing the descriptors. Of these, 113 were read in full and eventually, 16 articles matched the study design. The articles were published from 2016 to 2020, predominantly in English (87.5%). Concerning the main routes of administration in the studies included, nine addressed the intravenous infusion (in different modalities of time) and seven, subcutaneously. Conclusion: According to the scientific literature, all intravenous and subcutaneous infusion modalities are shown to be safe and effective if the protocols are correctly selected and applied

Introducción: Rituximab es un anticuerpo monoclonal quimérico de ratón/humano, ampliamente utilizado em el ámbito terapéutico de diversos diagnósticos. Debido a sus diferentes protocolos de administración, manejo y efectos adversos, su uso requiere la atención del equipo de salud. Objetivo: Describirlos protocolos de infusión de rituximab em la primera, subsiguiente y desensibilización, y caracterizar su seguridad. Método: Es una revisión integradora de la literatura. La búsqueda de revistas se realizó en bases de datos y bibliotecas electrónicas: LILACS, PubMed, MEDLINE, SciELO y BDEnf. Resultados: Cruzar los descriptores proporciono la elegibilidad de 413 artículos. De estos, 113 fueron leí dos íntegramente y al final, 16 artículos fueron aplicables al diseño del estudio. Los artículos fueron publicados entre 2016 y 2020, con predominio del inglés (87,5%). En cuanto a las principales formas de administración del fármaco, entre los estudios incluidos, nueve abordaron la infusión intravenosa (en diferentes modalidades de tiempo) y siete la vía subcutánea. Conclusión: Según la literatura científica, todas las modalidades de infusión intravenosa y subcutánea se muestran seguras y efectivas si los protocolos están debidamente indicados y correctamente aplicados

Blood glucose related adverse drug reaction of antitumor monoclonal antibodies: a retrospective analysis using Vigibase

Abstract On the increasing prevalence of using mAbs (monoclonal antibodies) in cancer therapy and the severe risk of hyperglycemia, we aimed to analyze the main clinical ADRs of mAbs, with a focus on adverse hyperglycemic events associated with currently clinically used mAbs. mAbs as well as target information were selected from Martinadale book and published articles. Drug approving information was collected from each government website, and ADR statistic data were collected from VigibaseR, comparing with Adverse Event Reporting System of US FDA. Top 10 mAbs were classified within listing in total ADR records, ADRs per year, hyperglycemic ADR records. Vigibase data were updated onto 15 Feb 2019. 20 mAbs were analyzed with 263217 ADR reports, wherein 16751 records on Metabolism and nutrition disorders and 1444 records on Glucose metabolism disorders. The geographic, age, gender distributions and annual ADR report numbers were listed respectively. Of the top 10, Rituximab, Bevacizumab and Nivolumab were on the top 3 in total ADR record and hyperglycemic record. Top 3 record results were similar in Vigibase and FDA database. It is of increasing importance for clinicians to be aware of early detection, patient management, or drug selection strategies when using mAbs, particularly within the high glycemic risk-reported mAbs, to improve the efficacy and tolerability of mAbs regiment and optimize patient outcomes.

Púrpura trombocitopénica idiopática (pti): su diagnóstico a través de lesiones en cavidad oral / Purple Idiopathic Thrombocytopenic (Itp): diagnosis through lesions in the oral cavity

La PTI es una alteración hemorrágica de instalación súbdita, adquirida, que se manifiesta inicialmente con petequias, equimosis o hematomas en piel y mucosas, sangrado nasal y gingival, sin causa aparente. La mucosa bucal puede ser el sitio donde las lesiones se observen con frecuencia y por primera vez. Se reporta el caso de un paciente masculino de 28 años de edad, con manifestaciones clínicas de un cuadro purpúrico, se describen signos, síntomas, terapéutica y manejo estomatológico (AU)

PTI is a hemorrhagic alteration of sudden installation, acquired, which manifests initially with petechiae, esquimosis or bruises on skin and mucosae, nasal and gingival bleeding without apparent cause. Bucal mucosae can be the site where lesions are observed with frequency, and for the first time. The case of a male patient with 28 years of age with clinical manifestationsofpurpuric syndrome is reported, signs, symptoms, therapeutic and stomatological handling are described (AU)

Linfoma folicular duodenal primario: reporte de caso y revisión de la literatura / Primary duodenal follicular lymphoma: Case report and literature review

Resumen Los linfomas primarios del tracto gastrointestinal son infrecuentes; sin embargo, son la presentación extranodal más común de los linfomas no Hodgkin. El 30 % de los linfomas no Hodgkin corresponde a linfomas foliculares y, a su vez, cerca del 10 % de los linfomas foliculares se origina en el tracto gastrointestinal. Se han descrito factores de riesgo para el desarrollo de linfomas gastrointestinales como infección por Helicobacter pylori, inmunosupresión posterior a trasplante de órganos sólidos, enfermedad inflamatoria intestinal e infección por virus de la inmunodeficiencia humana (VIH). El linfoma duodenal folicular se reconoció como una variante del linfoma folicular en 2016 según la clasificación de la Organización Mundial de la Salud (OMS), al considerar que se trata de una condición con características biológicas y clínicas particulares. Su diagnóstico suele ser incidental o se pueden presentar síntomas leves e inespecíficos. El grado histológico suele ser bajo y el curso clínico, benigno; por lo que en gran parte de los casos se ha adoptado el manejo expectante como una opción. Otras terapias con similar efectividad son la radioterapia, el uso de rituximab y la inmunoquimioterapia. No existe a la fecha suficiente evidencia para generar un protocolo único de manejo para esta patología.

Abstract Primary gastric lymphomas are rare diseases; however, they are the most common extranodal presentation of non-Hodgkin lymphomas. 30% of non-Hodgkin lymphomas correspond to follicular lymphomas and at the same time, nearly 10% of follicular lymphomas are produced in the gastrointestinal tract. Risk factors for gastric lymphomas such as Helicobacter pylori infection, immunosuppression after solid organ transplantation, inflammatory bowel disease, and human immunodeficiency virus (HIV) infection were described. Follicular duodenal lymphoma was recognized as a variant of follicular lymphoma in 2016 according to the World Health Organization (WHO) classification, considering that it is a condition with special biological and clinical characteristics. Its diagnosis is usually incidental or mild and nonspecific symptoms may occur. The histological grade is usually low, and the clinical course is benign; Therefore, in most cases, expectant treatment has been adopted as an option. Other therapies with similar effectiveness are radiotherapy, the use of rituximab, and immunochemotherapy. There is not enough evidence to date to generate a single management protocol for this pathology.

Eficacia y seguridad del rituximab en el tratamiento de la esclerosis múltiple: una postura racional en el contexto colombiano / Efficacy and safety of rituximab in the treatment of multiple sclerosis: a rational position in the Colombian context

RESUMEN INTRODUCCIÓN: La esclerosis múltiple es una enfermedad neuroinflamatoria, crónica, degenerativa e incurable, asociada a pérdida neuronal, grados crecientes de discapacidad y deterioro cognoscitivo. Su manejo conlleva grandes costos para los sistemas de salud y la sociedad en general. OBJETIVO: Evaluar la eficacia y la seguridad del uso del rituximab en el manejo de la esclerosis múltiple. MATERIALES Y MÉTODOS: Revisión de la literatura y evaluación de la tecnología en salud tipo mini-HTA en red colaborativa con el Comité de Esclerosis Múltiple de la Asociación Colombiana de Neurología y el Instituto Global de Excelencia Clínica. RESULTADOS: Se identificaron 27 referencias de texto completo para el análisis de la seguridad y la eficacia del rituximab en el manejo de la esclerosis múltiple. Se utilizaron análisis de costos, indicadores epidemiológicos y estudios pivótales de rituximab. CONCLUSIÓN: La evidencia analizada confirma que la terapia con rituximab es efectiva y segura en el manejo de las formas de esclerosis múltiple remitente-recurrente (EMRR) y esclerosis múltiple primaria-progresiva (EMPP), con menor tasa de eventos adversos y tasas de interrupción o abandono del tratamiento más bajas que otras terapias modificadoras de la enfermedad (TME).

SUMMARY INTRODUCTION: Multiple sclerosis is a neuroinflammatory, chronic, degenerative, and incurable disease, associated with neuronal loss, increasing degrees of disability, and cognitive control. Its treatment causes great costs for health systems and society in general. OBJECTIVE: To evaluate the efficacy and safety of the use of rituximab in the management of multiple sclerosis. MATERIALS AND METHODS: Literature review and evaluation of mini-HTA type health technology in a collaborative network with the Multiple Sclerosis Committee of the Colombian Association of Neurology and the Global Institute of Clinical Excellence. RESULTS: 27 full-text references were identified for the safety and efficacy analysis of rituximab in the management of multiple sclerosis. Cost analysis, epidemiological indicators, and pivotal studies of rituximab were incorporated into the analysis. CONCLUSION: The evidence analyzed confirms that rituximab therapy is effective and safe in the management of the forms of Recurrent-Remittent Multiple Sclerosis (RRMS) and Primary-Progressive Multiple Sclerosis (PPMS), with a lower rate of adverse events and discontinuation or withdrawal rates of treatment lower than other disease-modifying therapies.

Primary myocardial diffuse large B cell lymphoma: report of one case / Linfoma difuso de células grandes miocárdico primario: informe de un caso

ABSTRACT Primary myocardial involvement of Diffuse Large B-Cell lymphoma is extremely rare, accounting for 0.5 % of all lymphomas. We report a 65-year-old male, presenting with an acute cardiac tamponade, which was drained. A pericardial window with myocardial biopsy was carried out, disclosing a diffuse large B cell lymphoma. He received 6 cycles of rituximab, cyclophosphamide, vincristine, and prednisone (R-CVP), without response. Finally, a palliative chemotherapy with gemcitabine plus oxaliplatin was prescribed.

El linfoma difuso de células grandes B, primario del miocardio es muy raro. Presentamos un varón de 65 años que se presentó con un taponamiento cardíaco agudo que fue drenado. La biopsia miocárdica un mostró linfoma difuso de células grandes B, primario de miocardio. El paciente recibió 6 ciclos de quimioterapia con rituximab, ciclofosfamida, vincristina y prednisona sin respuesta. Finalmente se optó por una quimioterapia paliativa con gemcitabina y oxaliplatino.

Central nervous system international prognostic index impacts overall survival in diffuse large b-cell lymphoma treated with r-chop in a third level cancer center from Mexico: a survey of 642 patients

Background: Central nervous system international prognosis index (CNS-IPI) is validated in European and the USA cancer databases. However, no validation has been done in Mexican population. Objective: The objective of the study was to assess the impact of the CNS-IPI on central nervous system (CNS) relapse and survival in Mexican patients with diffuse large B-cell lymphoma (DLBCL). Methods: In this retrospective analysis, clinical, biochemical, and histological variables and the CNS-IPI were analyzed. Results: Six hundred and forty-two patients with DBLCL were included in the study. The mean ± SD age was 56.8 ± 14.9 years. Most had an ECOG of 0-1: 75% (n = 484) had absence of B-symptoms and advanced disease (clinical stage: III-IV, n = 433, 67.4%). According to the CNS-IPI, almost one-half were in the low-risk category. According to the CNS-IPI, CNS relapse rate was 1.36% (95% CI: 83.2-92.8), 3.1% (95% CI: 132.4-162.8), and 7.4% (95% CI 61-91) for patients in the low-, intermediate-, and high-risk categories, respectively. The median overall survival in the high-risk group (CNS-IPI) was 22 months, and it has not been achieved after 80 months of follow-up for the other groups. Conclusions: CNS-IPI was associated with survival; therefore, we propose its use as a prognostic tool for prospective validation.

Uso clínico do rituximabe em doenças autoimunes / Clinical use of rituximab for autoimmune diseases

Doenças autoimunes são doenças universais, e os diagnósticos e tratamentos primários são habitualmente iniciados por clínicos em enfermarias ou ambulatórios, antes de serem encaminhados a especialistas. Além disso, pacientes em uso de biológicos internados em hospitais gerais têm sido cada vez mais frequentes na prática clínica. Conhecer o perfil de segurança, as indicações e os efeitos colaterais dessas drogas deve ser preocupação dos clínicos. Neste trabalho, foi realizada revisão de literatura sobre terapia biológica com rituximabe no tratamento das principais doenças autoimunes sistêmicas da prática clínica: artrite reumatoide, lúpus eritematoso sistêmico, vasculites relacionadas aos anticorpos anticitoplasma de neutrófilo, púrpura trombocitopênica imune e espondilite anquilosante. (AU)

AutoimmunAutoimmune diseases are universal diseases and primary diagnosis and treatment are typically initiated by internists in wards or outpatient clinics before being referred to specialists. In addition, patients on use of biologicals hospitalized in general hospitals have been increasingly common in clinical practice. Knowing the safety profile, the indications, and the side effects of these drugs should be a concern for the internists as well. In this study, the literature review was performed on biological therapy with Rituximab for treating the main systemic autoimmune diseases of clinical practice: rheumatoid arthritis, systemic lupus erythematosus, anti-neutrophil cytoplasmic antibody-associated vasculitides, immune thrombocytopenic purpura, and ankylosing spondylitis. (AU)

Linfoma de Burkitt primario de mama durante el embarazo / Primary Burkitt's Lymphoma of the Breast During Pregnancy

El linfoma de Burkitt es una neoplasia altamente agresiva y es un tipo raro de linfoma no Hodgkin localizado. Aunque los niños son los más frecuentemente afectados, en adultos ocurren principalmente durante el embarazo o el puerperio. La mama rara vez constituye la localización primaria del linfoma no Hodgkin. Se presenta un caso de linfoma de Burkitt primario de mama durante el embarazo. Paciente de 37 años con embarazo de 24 semanas quien presentó aumento de volumen difuso de mama derecha. La mama estaba aumentada de tamaño, dolorosa y homogénea con tumoración elástica y firme. La ecografía demostró inflamación difusa con tumoración heterogénea e hipoecoica con contornos ligeramente irregulares, marcadores tumorales estaban normales las pruebas serológicas fueron negativas. La biopsia de la lesión mostró tejido mamario reemplazado por células linfoideas de tamaño mediano con citoplasma basófilo y múltiples vacuolas. Estudios inmunohistoquímicos fueron positivos para el antígeno leucocitario común, CD10, CD20, CD43, Bcl-6. El análisis cromosómico reveló que más del 90 % de las células neoplásicas exhibieron translocación t llevando al diagnóstico final de linfoma de Burkitt de mama. Luego de evaluar las posibilidades terapéuticas y del consentimiento de la paciente se inició tratamiento citostático sistémico. Los linfomas primarios de mama son extremadamente raros. El linfoma de Burkitt primario de la mama es mucho menos común que los otros linfomas. Los métodos de clasificación, detección y tratamiento de esta afección siguen siendo objeto de debates e investigaciones(AU)

The Burkitt's lymphoma is a highly aggressive neoplasm and is a rare type of localized non-Hodgkin lymphoma. Although children are the most frequently affected, in adults they occur mainly during the pregnancy or the puerperium. The breast rarely constitutes the primary location for non-Hodgkin lymphoma. The study of a case of primary Burkitt lymphoma of the breast during pregnancy is presented. This is a 37 year old patient with a 24 week pregnancy who presented a diffuse increase in the volume of the right breast. The breast was enlarged, painful and homogeneous with a firm, elastic mass. The ultrasonography showed diffuse inflammation with a heterogeneous and hypoechoic tumor with slightly irregular contours. The tumor marker values were normal and the serological tests were negative. The biopsy of the lesion showed breast tissue replaced by medium-sized lymphoid cells with basophilic cytoplasm and multiple vacuoles. Immunohistochemically studies were positive for the common leukocyte antigen, CD10, CD20, CD43, Bcl-6. The chromosomal analysis revealed that more than 90 % of neoplastic cells exhibited t translocation leading to the final diagnosis of Burkitt lymphoma of the breast. After evaluating the therapeutic possibilities and the patient's consent, systemic cytostatic treatment was started. Primary breast lymphomas are extremely rare. The primary Burkitt lymphoma of the breast is much less common than other lymphomas. The methods of classification, detection, and the treatment of this condition continue to be the subject of debate and research(AU)

Radiotherapy for early and advanced stages Follicular Lymphoma

OBJECTIVES To evaluate the results of radiotherapy (RT) for follicular lymphoma (FL) under different management scenarios. METHODS We retrospectively assessed consecutive patients with FL who had undergone irradiation between 2010 and 2018. All patients had biopsy-proven FL and were positron emission tomography-staged, although some (35.3%) were reassessed with computed tomography after treatment alone. Rituximab was only available to FL patients after 2016. RESULTS Thirty-four patients were selected, with a mean age at diagnosis of 61.6 years (34-89 years). The median follow-up duration was 49.4 months. Most patients were female (58.8%) and showed good performance on the Eastern Cooperative Oncology Group (ECOG) scale (ECOG 0-55.9%). The mean overall survival (OS) and progression-free survival were 48.7 and 33.6 months, respectively, with four deaths reported. OS rates at 2 and 3 years were 94.1% and 91.2%, respectively. Four patients showed transformation into aggressive lymphomas and underwent rituximab-based systemic treatment. Transformation-free survival was 47.8 months, and all patients with transformed disease were alive at assessment. Five patients had in-field relapse, all of them also relapsed elsewhere, and the mean relapse-free survival time was 40.3 months. No median end points were reached on assessment. CONCLUSION FL is an indolent disease. Our findings show good outcomes for patients treated with radiation, with a low transformation rate and excellent management of relapsed disease. RT is an important part of these results.

Is rituximab effective for systemic sclerosis? A systematic review and meta-analysis

Abstract Background: Systemic sclerosis (SSc) Is a clinically complex and challenging disease, that leads to skin fibrosis. Its most frequent complication is interstitial lung disease (ILD), which leads to a worse prognosis. In this situation, cyclophosphamide is considered the gold standard for its treatment, despite the controversies regarding its efficacy and toxicity. However, studies using rituximab (RTX) have shown that this drug may be a promising therapeutic option. Objectives: This paper objective was to analyze the scientific evidence on the RTX effects on SSc. Methods: A systematic review (SR) was performed including clinical trials (CTs) on the use of RTX in SSc, published up to May 2020. The studies were identified through systematic searches in bibliographic databases using a predefined search strategy. The following databases were used: PUBMED, SCOPUS, SCIELO, LILACS, SCIENCE DIRECT, WEB OF SCIENCE, COCHRANE, WHOLIS, PAHO and EMBASE. Also, a manual search was performed. The methodological quality of the studies was determined using Jadad scale, Risk of Bias Tool (RoB 2.0) and Risk of Bias in Non-Randomized Studies - of Interventions tool (ROBINS-I). A meta-analysis of the randomized CTs was performed, using Review Manager. Results: Ten CTs were included in this SR. Of these, three were randomized and seven were non-randomized. Five showed a statistically significant improvement in forced vital capacity (FVC) at some time during follow-up. Regarding the skin, eight studies showed statistically significant improvements according toa the modified Rodnan skin score. The meta-analysis found positive effects of RTX in SSc, with a statistical significance for lung disease. Conclusion: Rituximab is a promising strategy for the SSc-associated ILD and cutaneous fibrosis treatment. PROSPERO registration number: CRD42019132018.(AU)

Eficácia e segurança de Rituximabe comparado a outras drogas no tratamento de vasculites sistêmicas: revisão rápida de evidências / Efficacy and safety of Rituximab compared to other drugs in the treatment of systemic vasculitides: rapid response review of evidence

Objetivo: descrever as evidências disponíveis na literatura científica sobre eficácia e segurança do rituximabe comparado a diferentes tratamentos. Materiais e métodos: é uma revisão rápida de evidências científicas para tomada de decisão informada por evidências em políticas e práticas de saúde. Conclusão: o Rituximabe tem eficácia e segurança similares à da Ciclofosfamida, para terapia de indução de remissão e para manutenção da remissão e, para pacientes com doença recidivante, o Rituximabe é mais eficaz que a Ciclofosfamida para manter a remissão. Para terapia de manutenção, Rituximabe é mais eficaz que Azatioprina, com perfil de segurança similar. Diferentes regimes de dosagem do Rituximabe tem eficácia e segurança similar para terapia de manutenção. O Infliximabe parece ser superior ao Rituximabe nos desfechos de eficácia (indução e manutenção da remissão).

Objective: to describe the evidence available in the scientific literature on the efficacy and safety of rituximab compared to different treatments. Materials and Methods: is a rapid review of scientific evidence for evidence-informed decision making in health policy and practice. Conclusion: Rituximab has similar efficacy and safety to Cyclophosphamide, for remission induction therapy and for maintenance of remission, and for patients with relapsing disease, Rituximab is more effective than Cyclophosphamide in maintaining remission. For maintenance therapy, Rituximab is more effective than Azathioprine, with a similar safety profile. Different dosing regimens of Rituximab have similar efficacy and safety for maintenance therapy. Infliximab appears to be superior to Rituximab in efficacy outcomes (induction and maintenance of remission).