Linfocitosis monoclonal de células B en familiares de pacientes colombianos con síndromes linfoproliferativos crónicos B

Introducción. La linfocitosis monoclonal de células B, generalmente, precede la leucemia linfocítica crónica y afecta alrededor del 12 % de la población adulta sana. Esta frecuencia se incrementa en familiares de pacientes con síndromes linfoproliferativos crónicos de células B. Objetivo. Determinar la frecuencia de linfocitosis monoclonal B en familiares de pacientes con síndromes linfoproliferativos crónicos B, sus características inmunofenotípicas y citogenéticas, posible relación con agentes infecciosos, y seguimiento a corto plazo de población colombiana. Materiales y métodos. Se estudiaron 50 adultos sanos con antecedentes familiares de síndromes linfoproliferativos crónicos de célula B, empleando citometría de flujo multiparamétrica, pruebas citogenéticas y serológicas, encuesta de hábitos de vida y seguimiento a dos años. Resultados. La frecuencia encontrada de linfocitosis monoclonal B fue del 8 %, con predominio del sexo femenino y edad avanzada, incrementándose al 12,5 % en individuos con antecedentes familiares de leucemia linfocítica crónica. Tres de cuatro individuos presentaron inmunofenotipo de tipo leucemia linfocítica crónica, todas con bajo recuento. A su vez, en estos individuos se observa de manera significativa un mayor número de células/ µl en subpoblaciones linfocitarias T, junto con mayor predisposición a la enfermedad. Las poblaciones clonales descritas aumentan a lo largo del tiempo de manera no significativa. Conclusiones. La frecuencia y comportamiento de la linfocitosis monoclonal de célula B en pacientes con antecedentes familiares de síndromes linfoproliferativos crónicos B es similar a lo encontrado en estudios relacionados, lo que sugiere que no existe afectación de genes de mayor relevancia que puedan desencadenar una proliferación clonal descontrolada, pero que generan desregulación inmunológica que podría indicar un mayor riesgo de infección grave en estos individuos.

Introduction. Monoclonal B-cell lymphocytosis generally precedes chronic lymphocytic leukemia, affecting about 12% of the healthy adult population. This frequency increases in relatives of patients with chronic B-cell lymphoproliferative disorders. Objective. To determine the frequency of monoclonal B-cell lymphocytosis in relatives of patients with chronic B-cell lymphoproliferative disorders, their immunophenotypic/ cytogenetic characteristics, a possible relationship with infectious agents, and short-term follow-up in the Colombian population. Materials and methods. Fifty healthy adults with a family history of chronic B-cell lymphoproliferative disorders were studied using multiparametric flow cytometry, cytogenetic/serological testing, lifestyle survey, and 2-year follow-up. Results. The frequency of monoclonal B-cell lymphocytosis found was 8%, with a predominance of female gender and advanced age, increasing to 12.5% for individuals with a family history of chronic lymphocytic leukemia. Three out of four individuals presented chronic lymphocytic leukemia-type immunophenotype, all with low counts. In turn, a significantly higher number of cells/µΙ is observed in these individuals in T lymphocyte subpopulations, together with a greater predisposition to the disease. The described clonal populations increase over time in a non-significant manner. Conclusions. The frequency and behavior of monoclonal B-cell lymphocytosis in patients with family history of chronic B-cell lymphoproliferative disorders are like those found in related studies, which suggests that there is no involvement of more relevant genes that can trigger uncontrolled clonal proliferation, but that generates immunological deregulation that could justify a greater risk of serious infection in these individuals.

Acute lichenoid and varioliform pityriasis in a pediatric patient / Vasculitis linfocítica en pitiriasis liquenoide y varioliforme aguda de un paciente pediátrico

Abstract Background: Pityriasis lichenoides et varioliformis acuta (PLEVA) is a rare dermatosis recognized as a benign condition of unknown etiopathogenesis. It is more common in pediatric patients and young adults and is characterized by multiple small or large erythematous plaques spread over the trunk and extremities. Case report: We describe the case of a 5-year-old male, previously healthy, with multiple erythematous lesions that disappeared leaving hypopigmented macules. The biopsy reported histological changes suggestive of mycosis fungoides. After a second revision of lamellae in this hospital, lymphocytic vasculitis (LV) with focal epidermal necrosis consistent with acute pityriasis lichenoides (PL) was identified. Conclusions: The existing knowledge about PLEVA lacks a consensus in specifying its classification, etiopathogenesis, diagnosis, and treatment, so this clinical condition represents a medical challenge. The diagnosis is made by clinical suspicion and confirmed by histology. The objective of this article was to report a case of PLEVA with an atypical presentation due to its histopathological findings, being the first report showing LV in children, as well as a review of the literature.

Resumen Introducción: La pitiriasis liquenoide y varioliforme aguda (PLEVA) es una dermatosis poco frecuente, de etiopatogenia desconocida y evolución autolimitada. Es más común en pacientes pediátricos y adultos jóvenes, y está caracterizada por la presencia de múltiples placas eritematoescamosas pequeñas o grandes, diseminadas en el tronco y las extremidades. Caso clínico: Se describe el caso de un escolar de 5 años, de sexo masculino, previamente sano, que presentó múltiples cuadros de lesiones eritematosas que desaparecían dejando máculas hipopigmentadas. La biopsia reportó cambios histológicos sugestivos de micosis fungoide. Se realizó una segunda revisión de laminillas, identificando vasculitis linfocítica con necrosis epidérmica focal, consistente con pitiriasis liquenoide aguda. Conclusiones: El conocimiento acerca de la PLEVA carece de un consenso que especifique su clasificación, etiopatogenia, diagnóstico y tratamiento, por lo que esta condición clínica representa un desafío médico. El diagnóstico se realiza por sospecha clínica y se confirma por histología. El objetivo de este artículo fue reportar un caso de PLEVA con presentación atípica por los hallazgos histopatológicos, siendo este el primer reporte de vasculitis linfocítica en niños, y además se realiza una revisión de la literatura.

Asociación entre tiroiditis linfocítica crónica y carcinoma papilar de tiroides: Revisión sistemática y metaanálisis de estudios en especímenes quirúrgicos / Association between chronic lymphocityc thyroiditis and papillary thyroid carcinoma: Systematic review and meta-analysis of studieson surgical specimens

Introducción. Existen resultados inconsistentes con relación al planteamiento de la hipótesis que sugiere una mayor probabilidad de documentar un carcinoma papilar de tiroides en especímenes quirúrgicos con cambios compatibles con tiroiditis linfocítica crónica. En los metaanálisis existentes se han incluido estudios no comparables metodológicamente y no se proponen claras fuentes de sesgo, justificación para la realización del presente metaanálisis. Métodos. Se realizó una búsqueda bibliográfica en Pubmed y Embase. Fueron obtenidos estudios retrospectivos donde se comparaba la prevalencia de carcinoma papilar de tiroides en especímenes con y sin cambios por tiroiditis linfocítica crónica. La evidencia recolectada fue sintetizada estadísticamente. Resultados. Un total de 22 artículos fueron incluidos. La población estuvo conformada por 63.548 especímenes. El OR combinado fue 1,81 (IC95%: 1,51-2,21). Hubo heterogeneidad entre la distribución de las razones de oportunidad entre los estudios (I2= 91 %; p>0,00001). La forma del gráfico en embudo de los estudios incluidos en el análisis parece estar simétrica, lo que indica la ausencia del sesgo atribuible a los estudios pequeños. Conclusiones. La literatura actual sugiere que existe un mayor riesgo de documentar un carcinoma papilar de tiroides en especímenes quirúrgicos en los que se observan cambios compatibles con tiroiditis linfocítica crónica; sin embargo, existen fuentes de sesgo que no será posible controlar en estudios retrospectivos, por lo que recomendamos estudiar la hipótesis que sugiere una mayor probabilidad de diagnosticar un carcinoma papilar de tiroides en especímenes con cambios compatibles con tiroiditis linfocítica crónica mediante metodologías prospectivas

Introduction. Inconsistent results exist in the literature regarding the hypothesis statement suggesting an increased likelihood of documenting papillary thyroid carcinoma (PTC) in surgical specimens with changes compatible with chronic lymphocytic thyroiditis. Existing meta-analyses have included studies that are not methodologically comparable and do not propose clear sources of bias, thus, this is justification for the present meta-analysis. Methods. A literature search in Pubmed and Embase was performed from January 1, 1950 to December 31, 2020. Retrospective studies comparing the prevalence of papillary thyroid carcinoma in specimens with and without chronic lymphocytic thyroiditis changes were obtained. The collected evidence was statistically analyzed. Results. A total of 22 articles were included. The study population consisted of 63,548 surgical specimens. The pooled OR, based on the studies, was 1.81 (95% CI: 1.51-2.21). There was heterogeneity between the distribution of prevalence ratios and opportunity ratios across studies (I²= 91%; p>0.00001). The funnel plot shape of the studies included in the analysis appears to be symmetrical, indicating the absence of bias attributable to small studies. Conclusions. The current literature suggests that there is an increased risk of documenting papillary thyroid carcinoma in surgical specimens in which chronic lymphocytic thyroiditis-compatible changes are observed; however, there are sources of bias that will not be possible to control for in retrospective studies, so we recommend studying the hypothesis suggesting an increased likelihood of diagnosing PTC in specimens with chronic lymphocytic thyroiditis-compatible changes using prospective methodologies

Radioterapia en pacientes pediátricos con leucemia linfocítica aguda: experiencia de 9 años

Objetivo: Describir las características epidemiológicas y clínicas de los pacientes con leucemia linfocítica aguda atendidos en el Hospital Nacional de Niños "Dr. Carlos Sáenz Herrera", que recibieron radioterapia externa, durante el periodo de enero de 2009 a diciembre de 2017. Métodos: Estudio observacional, descriptivo. Se revisaron retrospectivamente los expedientes clínicos de pacientes pediátricos (0-13 años) con leucemia linfocítica aguda, que recibieron radioterapia externa en el periodo mencionado. Se aplicó un análisis estadístico descriptivo de las variables cualitativas y cuantitativas. Resultados: Se analizó un total de 58 pacientes, de estos el 79,3% fueron hombres. La edad promedio fue de 7,3 años. El 84,2% fueron clasificados como L1, 84,2% con inmunofenotipo B común y el 56,9% eran grupo de alto riesgo al diagnóstico. La principal indicación de radioterapia fue recaída (67,7%). Aproximadamente la mitad se irradió a sistema nervioso central y la otra mitad a testículos. Los principales efectos adversos fueron cutáneos. Conclusiones: Los resultados obtenidos fueron comparables con los reportados en la literatura. La radioterapia es importante en el tratamiento de leucemias, especialmente en pacientes de recaída y de alto riesgo.

Aim: To describe the epidemiological and clinical characteristics of patients with acute lymphocytic leukemia, attended at the National Children´s Hospital "Dr. Carlos Sáenz Herrera" that received external radiation therapy between January 2009 and December 2017. Methods: It is an observational, descriptive study. Clinical records of pediatric patients (0-13 years) with acute lymphoblastic leukemia that received external radiotherapy in the study period were retrospectively reviewed. A descriptive statistical analysis of the qualitative and quantitative variables was applied. Results: 58 patients were studied, 79,3% were males. The mean age was 7,3 years. 84,2% were classified as L1, 84,2% had common B immunophenotype and 56,9% were in the high risk group at diagnosis. The main indication for radiotherapy was relapse (67,7%). About half the patients received radiotherapy to central nervous system and the other half to testicles. The main side effects were cutaneous. Conclusions: The results obtained were comparable to those seen in literature. Radiotherapy is important in leukemia treatment, particularly in relapse and high risk patients.

Leucemia linfocítica aguda con infiltración en la piel. Presentación de un caso y revisión de la literatura

RESUMEN La leucemia cutánea es una patología muy poco frecuente y se puede presentar en diferentes tipos de leucemias asociadas o no a síndromes genéticos. Es una forma muy poco común de presentación inicial de malignidad. Por sus lesiones inespecíficas en la piel y la similitud con diversas patologías cutáneas representa un gran desafío diagnóstico para el dermatólogo pediátrico. Se presenta el caso de un niño de 5 años con antecedentes de leucemia linfocítica aguda con lesiones papulosas descamativas de aspecto liquenoide diseminadas en tegumento cutáneo. Se realizó una biopsia bajo la sospecha de infiltración a piel o leucemia cutis. Se discutió el caso para llegar a la confirmación diagnóstica de leucemia cutánea. Se inició el tratamiento en el Servicio de Hematología. Esta enfermedad hematológica se manifiesta ocasionalmente en la piel y cuando lo hace es necesario reconocerla para completar su diagnóstico y tratamiento y salvar la vida del paciente afectado como en este caso. El interés de esta presentación radica en que la aparición de lesiones cutáneas, aunque sean inespecíficas, en un paciente con leucemia, debe alertar al equipo médico tratante para su rápido estudio y así orientar la conducta terapéutica y por su baja incidencia de presentación.

ABSTRACT Cutaneous leukemia is a very rare pathology and can occur in different types of leukemia associated or not with genetic syndromes. It is a very uncommon form of initial presentation of malignancy. Due to its non-specific skin lesions and the similarity with various skin pathologies, it represents a great diagnostic challenge for the pediatric dermatologist. We present the case of a 5-year-old boy with a history of acute lymphocytic leukemia with scaly papular lesions of a lichenoid appearance disseminated in the cutaneous integument. A skin biopsy was performed on suspicion of skin infiltration or leukemia cutis. The case was discussed to reach diagnostic confirmation of cutaneous leukemia. Treatment was started in the Hematology Service. This hematological disease occasionally manifests itself on the skin and when it does, it is necessary to recognize it to complete its diagnosis and treatment and save the life of the affected patient, as in this case. The interest of this presentation lies in the fact that the appearance of skin lesions, even if they are non-specific, in a patient with leukemia, should alert the treating medical team for their rapid study and thus guide therapeutic behavior and due to their low incidence of presentation.

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.

Factores de riesgo asociados a pacientes con colitis microscópica / Risk factors associated with patients with microscopic colitis / Fatores de risco associados a pacientes com colite microscópica

RESUMEN Introducción: La primera descripción de la colitis microscópica se realizó en el año 1976. Actualmente, agrupa tres subgrupos de patologías, las clásicas colitis linfocítica y colitis colagenosa, el tercer subgrupo corresponde a la colitis microscópica incompleta. Objetivo: Identificar los principales factores asociados al desarrollo de colitis microscópica en el Hospital General Provincial ¨Carlos Manuel de Céspedes¨, de Bayamo, Granma, en el período marzo de 2019 hasta agosto de 2021. Método: Se realizó un estudio observacional, analítico de casos y controles en pacientes con edad igual o mayor de 18 años con diarreas acuosas crónicas atendidos en el servicio de gastroenterología de dicho hospital, donde se estudiaron las variables: presencia de colitis microscópica, edad, sexo, hábito de fumar, uso de medicamentos, comorbilidades, dolor abdominal, fatiga, incontinencia fecal, pérdida de peso. El tamaño de la muestra para estudios pareados se determinó mediante el programa STATA 17. Para su estimación se consideró lo siguiente: nivel de confianza, poder del estudio, relación de casos y testigos y Odds Ratio mínimo. Resultados: No existió asociación entre la edad mayor de 50 años y la probabilidad de presentar colitis. Predominó el sexo femenino (62,5 %). Del total de pacientes con colitis microscópica (n=16) el 62,5 % fumaba. El OR obtenido indicó que los pacientes fumadores tienen 2,5 veces más riesgo. La diabetes mellitus se asoció significativamente al diagnóstico de colitis microscópica y quintuplicó el riesgo. Conclusiones: Existe una relación entre el sexo femenino, el hábito de fumar, la diabetes mellitus, la colecistectomía, el consumo de tres o más fármacos y la incontinencia fecal con la presencia de colitis microscópica.

ABSTRACT Introduction: The first description of microscopic colitis was made in 1976. Currently, it groups three subgroups of pathologies, the classic lymphocytic colitis and collagenous colitis, the third subgroup corresponds to incomplete microscopic colitis. Objective: To identify the main factors associated with the development of microscopic colitis at the Hospital General Provincial "Carlos Manuel de Céspedes", Bayamo, Granma, from March 2019 to August 2021. Method: An observational, analytical study of cases and controls was carried out in patients aged 18 years or older with chronic watery diarrhea treated at the gastroenterology service. The variables studied were: presence of microscopic colitis, age, sex, smoking habit, use of medications, comorbidities, abdominal pain, fatigue, fecal incontinence and weight loss. The sample size for paired studies was determined using the STATA 17 program. For its estimation, the following were considered: confidence level, statistical power of the test, ratio of cases and controls and minimum Odds Ratio. Results: There was no association between age over 50 years and the probability of presenting colitis. The female sex prevailed (62.5%). Of the total number of patients with microscopic colitis (n=16), 62.5% smoked. The OR obtained indicated that smoker patients have 2.5 times more risk. Diabetes mellitus was significantly associated with the diagnosis of microscopic colitis and increased the risk fivefold. Conclusions: There is a relationship between female sex, smoking, diabetes mellitus, cholecystectomy, consumption of three or more drugs and fecal incontinence with the presence of microscopic colitis.

RESUMO Introdução: A primeira descrição da colite microscópica foi feita em 1976. Atualmente, agrupa três subgrupos de patologias, a colite linfocítica clássica e a colite colagenosa, o terceiro subgrupo corresponde à colite microscópica incompleta. Objetivo: Identificar os principais fatores associados ao desenvolvimento de colite microscópica no Hospital Geral Provincial ¨Carlos Manuel de Céspedes¨, em Bayamo, Granma, de março de 2019 a agosto de 2021. Método: Estudo observacional analítico de casos e controles em pacientes com 18 anos ou mais com diarreia aquosa crônica atendidos no serviço de gastroenterologia do referido hospital, onde foram estudadas as variáveis: presença de colite microscópica, idade, sexo, tabagismo, uso de medicamentos, comorbidades, dor abdominal, fadiga, incontinência fecal, perda de peso. O tamanho da amostra para estudos pareados foi determinado por meio do programa STATA 17. Para sua estimativa foram considerados: nível de confiança, poder do estudo, razão de casos e controles e Odds Ratio mínimo. Resultados: Não houve associação entre idade acima de 50 anos e probabilidade de apresentar colite. O sexo feminino prevaleceu (62,5%). Do total de pacientes com colite microscópica (n=16), 62,5% fumavam. A OR obtida indicou que pacientes tabagistas apresentam risco 2,5 vezes maior. O diabetes mellitus foi significativamente associado ao diagnóstico de colite microscópica e aumentou o risco em cinco vezes. Conclusões: Existe relação entre sexo feminino, tabagismo, diabetes mellitus, colecistectomia, consumo de três ou mais medicamentos e incontinência fecal com a presença de colite microscópica.

Microscopic colitis: Case series and literature review / Colitis microscópica, serie de casos y revisión de la literatura

Abstract Introduction: Microscopic colitis is a benign and multifactorial disease characterized by watery diarrhea and histological alterations in the colonic mucosa. The incidence of this disease is increasing, being diagnosed more frequently. Materials and methods: In this retrospective study, patients were examined employing colonoscopy and biopsy due to a diagnosis of chronic diarrhea in a gastroenterology unit throughout 22 months. Their diagnosis of colitis was confirmed by clinical picture and microscopic analysis. Results: In the study period, a total of 2849 colonoscopies were performed, 116 in patients with chronic diarrhea. We identified 15 patients with microscopic colitis, 12 were men (80 %), and only three were older than 60 (20 %). Conclusion: Unlike the world literature, this study found that microscopic colitis in our patients affects the male sex primarily (male/female ratio: 4/1) and occurs in young people, with an average age of 47.5 years (range: 21-82 years).

Resumen Introducción: la colitis microscópica es una enfermedad benigna y multifactorial caracterizada por la presencia de diarrea acuosa y alteraciones histológicas en la mucosa colónica. La incidencia de esta enfermedad viene en aumento y su diagnóstico se realiza cada vez con mayor frecuencia. Métodos: estudio retrospectivo en el que se revisaron los pacientes por medio de colonoscopia y biopsias por diagnóstico de diarrea crónica en un período de 22 meses en una unidad de gastroenterología, y en quienes mediante la clínica y el análisis histológico se confirmó el diagnóstico de colitis microscópica. Resultados: en el período de estudio se realizó un total de 2849 colonoscopias, 116 en pacientes con diarrea crónica. Se identificaron 15 pacientes con colitis microscópica, 12 fueron hombres (80 %) y solo hubo 3 mayores de 60 años (20 %). Conclusión: a diferencia de lo informado en la literatura mundial, en este estudio se encontró que la colitis microscópica en nuestros pacientes compromete especialmente al sexo masculino (relación hombre/mujer: 4/1) y se presenta en personas jóvenes, con un promedio de edad de 47,5 años (rango de 21 a 82 años).

Blinatumomabe em pacientes pediátricos com leucemia linfoblástica aguda B em primeira recidiva de alto risco: um estudo de custo-efetividade / Blinatumomab in high-risk first relapse pediatric patients with acute lymphoblastic leukemia: a cost-effectiveness analysis

Objetivo: Estimar a custo-efetividade do blinatumomabe como novo padrão no tratamento de consolidação de pacientes pediátricos com leucemia linfoblástica aguda de células precursoras B (LLA-B) em primeira recidiva de alto risco. Métodos: Um modelo de sobrevida particionado com horizonte lifetime e ciclo de quatro semanas foi construído na perspectiva do Sistema Único de Saúde (SUS). Sobrevida livre de eventos e sobrevida global foram extrapoladas com base no ensaio clínico 20120215, usando funções paramétricas. A taxa de desconto foi de 5%. O impacto de variações em pressupostos foi explorado em análises de cenário. Resultados: O custo lifetime com desconto para o caso base foi de R$ 351.615 para blinatumomabe contra R$ 97.770 para HC3 (grupo controle de quimioterapia-padrão), com ganho de 9,96 e 6,74 anos de vida ajustados para qualidade (QALYs), respectivamente. A razão de custo-efetividade incremental (RCEI) foi de R$ 78.873/QALY. Considerando um cenário sem descontos, a RCEI foi de R$ 33.731/QALY ganho. Os outros cenários com maior impacto na RCEI foram a exclusão do desperdício de blinatumomabe (isto é, considerando que a sobra em frasco-ampola de um paciente seria reaproveitada para outro paciente: R$ 35.751) e a alteração do tempo de infusão (troca de bolsa em 48 ou 96 horas em vez de 24 horas: R$ 35.515). A probabilidade de o blinatumomabe ser custo-efetivo foi de 65,7% na análise probabilística, considerando um limiar de R$ 95.501. Conclusões: Blinatumomabe é custo-efetivo para pacientes pediátricos com LLA-B derivada em primeira recidiva de alto risco na perspectiva do SUS.

Objective: To estimate the cost-effectiveness of blinatumomab as the new standard treatment of consolidation in high-risk first relapse pediatric patients with B-cell acute lymphoblastic leukemia (B-ALL). Methods: A partitioned survival model with a lifetime horizon and a 4-week cycle was developed from the Brazilian public healthcare payer's perspective (SUS). Event-free survival and overall survival were extrapolated based on data from the 20120215 clinical trial using parametric functions. A 5% discount rate was used, and the impact of variations in model parameters and assumptions were explored in scenario analyses. Results: The discounted base case lifetime cost was R$ 351,615 for blinatumomab vs. R$ 97,770 for standard chemotherapy control group (HC3), with 9.96 QALYs gained with blinatumomab vs. 6.74 QALYs gained with HC3. The incremental costeffectiveness ratio (ICER) was R$ 78,873/QALY. Considering an undiscounted scenario, the ICER was.

Análise de custo do sequenciamento dos novos agentes para o tratamento de leucemia linfocítica crônica sob a perspectiva do sistema de saúde suplementar brasileiro / Cost analysis of sequencing new agents for the treatment of chronic lymphocytic leukemia from the perspective of the Brazilian supplementary health system

Objetivo: Estimar o custo do sequenciamento de tratamentos e por desfecho dos novos agentes disponíveis para o tratamento de pacientes com leucemia linfocítica crônica (LLC) em primeira linha (1L) e segunda linha (2L) em um horizonte temporal de 15 anos sob a perspectiva do sistema de saúde suplementar brasileiro. Métodos: Foi desenvolvido um modelo de sobrevida particionada com quatro transições de estados de saúde (sem progressão em 1L, sem progressão em 2L, pós-progressão e morte), considerando os seguintes regimes: venetoclax + obinutuzumabe (VenO), venetoclax + rituximabe (VenR), ibrutinibe (Ibru) e acalabrutinibe (Acala). Foram consideradas na análise as posologias em bula e as curvas de sobrevida livre de progressão (SLP) dos respectivos estudos pivotais em cada uma das linhas terapêuticas. O custo total de cada sequência considerou a soma dos custos dos regimes utilizados em 1L e 2L, baseado no preço fábrica de cada medicamento. Resultados: As sequências de tratamento iniciadas com VenO apresentaram menores custos, especialmente o regime VenO>VenR (R$ 982.447), que apontou redução de aproximadamente R$ 3 milhões em 15 anos, quando comparada às sequências de Ibru>VenR ou Acala>VenR. Na análise de custo por desfecho, a sequência VenO>VenR apresentou o menor custo por ano de SLP (R$ 104.437), até 76% inferior em relação ao sequenciamento com maior custo por ano de SLP (Ibru>VenR). Conclusões: Os resultados desta análise demonstram o impacto significativo que a 1L de tratamento possui na jornada do paciente com LLC. Adicionalmente, o presente estudo aponta o menor custo de tratamento acumulado para o sequenciamento dos regimes VenO>VenR, sugerindo que os regimes de tratamento à base de venetoclax podem contribuir de maneira substancial em uma maior eficiência na alocação de recursos pelo gestor do sistema de saúde suplementar brasileiro.

Objective: To estimate the cost of treatment sequencing and per outcome of the new agents available for the treatment of patients with chronic lymphocytic leukemia (CLL) in 1st line (1L) and 2nd line (2L) in a 15-years time horizon from the perspective of the Brazilian supplementary health system. Methods: A partitioned survival model including four health state transitions (no progression in 1L, no progression in 2L, post-progression and death) was developed, considering the following regimens: venetoclax + obinutuzumab (VenO), venetoclax + rituximab (VenR), ibrutinib (Ibru) and acalabrutinib (Acala). The package insert dosages and progression-free survival (PFS) curves of the respective pivotal studies in each of the therapeutic lines were considered in the analysis. The total cost of each sequence considered the sum of the costs of the regimens used in 1L and 2L, based on the factory price of each drug. Results: Lower costs were observed when treatment sequences were initiated with VenO, especially the VenO>VenR regimen (R$ 982,447), which showed a reduction of approximately R$ 3 million in 15 years when compared to the Ibru>VenR or Acala>VenR sequences. In the cost per outcome analysis, the sequence VenO>VenR had the lowest cost per year of PFS (R$ 104,437), up to 76% lower than the sequencing with the highest cost per year of PFS (Ibru>VenR). Conclusions: Results show the significant impact that 1L treatment has on the CLL patient's journey. Additionally, the present study points to the lowest accumulated treatment cost for the sequencing of VenO>VenR regimens, suggesting that venetoclax-based treatment regimens can substantially contribute to greater efficiency in the allocation of resources by the manager of the Brazilian supplementary health system.

Leucemia linfocítica crônica associada a melanoma metastático em homem de 53 anos / Metastatic melanoma in a 53-year-old male with relapsed chronic lymphocytic leukemia

Introdução: A associação de leucemia linfocítica crônica (LLC) e melanoma tem sido estudada nos últimos anos. Acredita-se que a imunossupressão causada pelo tratamento da doença seja o fator de risco mais importante para o aumento da susceptibilidade ao desenvolvimento e disseminação do câncer de pele. Relato de Caso: Este relato de caso descreve homem de 53 anos, em tratamento de leucemia linfocítica crônica desde 2018, já submetido a diversos ciclos de quimioterapia com fludarabina. Apresentou histórico de exérese de melanoma nodular epitelioide no couro cabeludo em 2019, removido com margens livres. Um ano após a cirurgia, paciente evoluiu com piora do estado geral com necessidade de hospitalização. Investigação adicional revelou focos de metástase em pulmões, fígado, rins, estômago, sistema nervoso central e linfonodos. Análise histopatológica foi positiva para melanoma. A possibilidade de tratamento foi descartada pela equipe de oncologia, que sugeriu cuidados paliativos. Discussão: Um dos mecanismos mais discutidos para explicar esta associação de neoplasias é a imunossupressão resultante do tratamento da LLC, que deixa o paciente suscetível ao desenvolvimento e à disseminação do melanoma. Além disso, a fludarabina, quimioterápico geralmente usado para remissão da LLC, é conhecida por depletar células T-helper e tem sido descrita como cofator deste processo. A associação de leucemia e melanoma cutâneo têm sido descrita nos últimos anos, porém não há nenhum protocolo de tratamento para esta condição.

BACKGROUND: The association of Chronic Lymphocytic Leukemia (CLL) and melanoma have been studied in the last years. The immunosuppression caused by the treatment of CLL seems to be the major factor of increasing patients' susceptibility to the development and spread of skin cancer. CASE REPORT: This case report describes a 53-year-old male patient, in CLL treatment since 2018, already submitted to many cycles of chemotherapy with fludarabine. History of an exeresis of epithelioid nodular melanoma of the scalp in 2019, which was removed with a clear margin. One year later, he presented with a poor general condition with hospitalization indication. Additional investigation revealed metastatic lesions in lungs, liver, kidneys, stomach, central nervous system, and lymph nodes. Histopathologic analysis positive for melanoma. The possibility of treatment was discarded by the Oncology team, which suggested palliative care. DISCUSSION: One of the most discussed mechanisms to explain this cancer association is the immunosuppression developed during the treatment of CLL, increasing patients' susceptibility to the development and spread of melanoma. In addition, the use of fludarabine, a chemotherapy commonly used in relapsed CLL, is known to deplete T-helper cells and has been described as a cofactor of this process. The association of leukemia and cutaneous melanoma has been reported in the last years, yet there is no surveillance protocol.

Leucemia linfocítica crónica y deleción del gen TP53: reporte de un caso y revisión de la literatura / Chronic lymphocytic Leukemia and deletion of the TP53 gene: Case report and literature review

RESUMEN Las alteraciones genéticas en el gen TP53 están presentes entre el 5 al 8 % de los pacientes de leucemia linfocítica crónica (LLC) en el momento del diagnóstico. Estos casos se relacionan con un mal pronóstico debido a su resistencia al tratamiento estándar. Presentamos el caso de un paciente masculino de 52 años diagnosticado con LLC, expresión del marcador CD38 y una deleción en el gen TP53 (17p13.1). Tras la evaluación posterior del tratamiento, se observó enfermedad mínima residual lo que llevó a un trasplante haploidéntico de progenitores hematopoyéticos. Debido al alto riesgo de recaída, su edad y la ausencia de comorbilidades, era la única opción curativa hasta la fecha para la LLC. El objetivo de este trabajo es realizar una revisión de la literatura que sirva como base para analizar el caso clínico presentado, en el marco de las implicaciones clínicas, pronóstico y respuesta al tratamiento en los individuos con LLC que presentan alteraciones en el gen TP53.

SUMMARY Genetic alterations in the TP53 gene are present in 5 to 8% of chronic lymphocytic leukemia (CLL) cases at the time of diagnosis. These cases are typically associated with poor prognosis due to their resistance against standard CLL treatment. In our report a 52-yearold male patient was diagnosed with CLL, CD38 expression and a deletion in the TP53 gene (17p13.1). Upon evaluation post-treatment, minimal residual disease (MDR) was observed, and a haploidentical stem cell transplant was performed. Because of the high risk of relapse, his age, and the absence of comorbidities it was the only curative option to date for CLL. The purpose of this article is to complete a literature review that will give a basis to analyze the clinical case presented, within the framework of the clinical implications, prognosis, and response to treatment in patients with CLL who present with aberrations of the TP53 gene.

Injuria subendocárdica sin evidencia de aterosclerosis relacionada con infusión de obinutuzumab / Subendocardial injury with no evidence of atherosclerosis associated with obinutuzumab infusión

Resumen El obinutuzumab es un anticuerpo monoclonal completamente humanizado contra CD20, empleado en el tratamiento de leucemia linfocítica crónica. Los eventos cardiovasculares fatales han sido des critos, pero solo en pacientes con antecedentes cardiovasculares conocidos. Presentamos el caso de un hombre adulto con diagnóstico de leucemia linfocítica crónica de alto riesgo que desarrolló injuria subendocárdica, sin evidencia de aterosclerosis coronaria, durante la primera infusión de obinutuzumab.

Abstract Obinutuzumab is a fully humanized monoclonal antibody against CD20 used in the treat ment of chronic lymphocytic leukemia. Fatal cardiovascular events have been described, but only in patients with known cardiovascular records. We report the case of an adult male with a high-risk chronic lymphocytic leukemia who developed subendocardial injury, with no evidence of coronary atherosclerosis, during the first administration of obinutuzumab.

Linfoma Hodgkin Clásico en el departamento de Anatomía Patológica del Hospital Nacional Edgardo Rebagliati Martins durante los años 2015 a 2019 / Classic Hodgkin Lymphoma at the Edgardo Rebagliati Martins National Hospital during 2015 to 2019

RESUMEN Introducción: Los Linfomas Hodgkin son neoplasias linfoides de células B, caracterizadas histológicamente por un contexto celular inflamatorio mixto mayoritario y escasas células neoplásicas de Hodgkin/ Reed- Sternberg. El Linfoma Hodgkin Clásico (LHC) representa el 10% de todos los casos de linfoma y el 85% de todos los Linfomas Hodgkin. De acuerdo con la vigente clasificación de la Organización Mundial de la Salud, el LHC se divide en 4 variantes: Esclerosis Nodular (EN), Celularidad Mixta (CM), Rico en Linfocitos (RL) y Depleción Linfocítica (DL). Objetivo: En este estudio revisamos todos los casos de Linfoma Hodgkin Clásico en el Departamento de Anatomía Patológica del Hospital Nacional Edgardo Rebagliati Martins durante los años 2015 a 2019, para determinar la variante más frecuente, la incidencia en cuanto a edad y sexo, características fenotípicas y relación con el Epstein Barr Virus (EBV). Materiales y Métodos: Se realizó un estudio descriptivo retrospectivo de la casuística de Linfoma Hodgkin Clásico en sus 4 variantes clínico - patológicas en el Departamento de Anatomía Patológica del Hospital Nacional Edgardo Rebagliati Martins durante los años 2015 a 2019. Se identificaron 72 pacientes con el diagnóstico de Linfoma Hodgkin Clásico, de los cuales únicamente se seleccionaron para el estudio 64. Los criterios de exclusión fueron la ausencia de pruebas de inmunohistoquímica confirmatoria y los casos de recidiva. Resultados: Se observó que la variante más frecuente correspondió a Esclerosis Nodular con 34 casos (53.12%) y la menos frecuente a la variante Rica en Linfocitos con 2 casos (3.12%). Así mismo se observó una predominancia en el sexo masculino con 42 casos, 20 de ellos con Esclerosis Nodular y 14 no clasificables, como las variantes más frecuentes, y una mayor incidencia entre los 41 y 50 años de edad, sin detectarse el pico bimodal referido en la literatura internacional. El perfil inmunohistoquímico más frecuente de las células Hodgkin/ Reed- Sternberg es CD15 y CD30 positivo, con CD45 negativo. El EBV estuvo presente en el 36% de los casos realizados y es más frecuente en las variedades Celularidad Mixta y Depleción Linfocítica. Conclusiones: El Linfoma Hodgkin Clásico es un grupo de neoplasias linfoides con características clínicas, histológicas y fenotípicas definidas. Es más frecuente en varones entre 41 y 50 años. Para un adecuado diagnóstico se requiere una completa información clínica y una buena biopsia, de preferencia excisional. La variante Esclerosis Nodular es la más frecuente y la Rica en Linfocitos la menos frecuente. Las células Hodgkin/ Reed- Sternberg suelen ser positivas para CD15 y CD30 y negativas para CD45. La positividad tenue del Pax-5 permite diferenciarlo de Linfomas no Hodgkin de Células B. El EBV es más frecuente en las variantes Celularidad Mixta y Depleción Linfocítica.

ABSTRACT Introduction: Hodgkin lymphomas are B-cell lymphoid neoplasms histologically characterized by a mixed inflammatory cellular component and few Hodgkin/Reed-Sternberg neoplastic cells. Classical Hodgkin Lymphoma (CHL) represents 10% of all lymphoma cases and 85% of all Hodgkin Lymphomas. According to the current World Health Organization classification, CHL is divided into 4 types: Nodular Sclerosing (NS), Mixed Cellularity (MC), Lymphocyte-Rich (LR), and Lymphocyte-Depleted (LD). Objective: We reviewed all cases of Classical Hodgkin Lymphoma in the Pathological Anatomy Department at Edgardo Rebagliati Martins National Hospital during 2015 to 2019, in order to determine the most frequent type, the incidence according to age and gender, phenotypical characteristics and relation to Epstein Barr Virus (EBV). Materials and Methods: We performed a retrospective descriptive case study of Classical Hodgkin Lymphoma and its 4 clinical-pathological types in the Pathological Anatomy Department at Edgardo Rebagliati Martins National Hospital during 2015 to 2019. 72 patients were identified with Classical Hodgkin Lymphoma diagnosis, of which only 64 were selected for the study. The exclusion criteria were the absence of confirmatory immunohistochemical tests and relapse cases. Results: The most frequent type observed was Nodular Sclerosing with 34 cases (53.12%) and the least frequent type was Lymphocyte-Rich with 2 cases (3.12%). Likewise, a predominance in the male gender was observed, with 42 cases, 20 of which were Nodular Sclerosing and 14 not classified, as the most frequent types, and a greater incidence among those 41 to 50 years of age, without detection of the bimodal peak referenced in international literature. The most frequent immunohistochemical profile of Hodgkin/ Reed- Sternberg was CD15 and CD30 positive, with CD45 negative. EBV was present in 36% of cases and is more frequent in the Mixed Cellularity and Lymphocyte-Depleted types. Conclusions: Classical Hodgkin Lymphoma is a group of lymphoid neoplasms with clinical, histological, and phenotypically defined characteristics. It is more frequent in men between 41 and 50 years of age. A complete clinical information and a good biopsy, preferably excisional, is required for an adequate diagnosis. The Nodular Sclerosing type is the most frequent and the Lymphocyte-Rich is the least frequent type. Hodgkin/ Reed- Sternberg cells are usually CD-15 and CD-30 positive and CD-45 negative. The Pax-5 mild positivity allows it to be differentiated from B-cell Non-Hodgkin Lymphomas. EBV is most frequent in Mixed Cellularity and Lymphocyte-Depleted types.

Alterações retinianas em pacientes com leucemia linfocítica aguda (lla): discussão de tema a partir de relato de caso / Retinal changes in patients with acute lymphocytic leukemia (all): discussion of topic from a case report

RESUMO A Leucemia Linfocitica Aguda (LLA) é uma doença caracterizada por uma alta taxa de sobrevida, porém o número absoluto de crianças que morrem por ela representa uma grande parcela dos casos de óbitos infantis por câncer. A morbidade decorrente de seu tratamento pode deixar sequelas em pessoas com grande expectativa de vida, tornando-se extremamente necessário o entendimento da patogênese desta doença, possibilitando o desenvolvimento de novos tratamentos e diminuição de sequelas provocadas pela doença. O diagnóstico precoce é importante para se evitar complicações oculares que possam levar a baixa de acuidade visual em longo prazo e para avaliação de recaídas de tratamento sendo determinante no direcionamento de condutas.

ABSTRACT The Acute Lymphocytic Leukemia (ALL) is a disease characterized by a high survival rate, but the absolute number of children who die from it represents a large proportion of cases of infant deaths from cancer. The morbidity resulting from its treatment can leave sequelae in people with high life expectancy, making it extremely necessary to understand the pathogenesis of this disease, enabling the development of new treatments and reduction of sequelae caused by the disease. This early diagnosis is important to avoid ocular complications that may lead to low long-term visual acuity and to evaluate treatment relapses and determine the conducts.

Leucemias crónicas y SARS-CoV-2 / Chronic leukemias and SARS-CoV-2

Resumen El 30 de enero del año 2020, la Organización Mundial de la Salud declaró emergencia internacional de Salud Pública la pandemia causada por el coronavirus 2 del síndrome respiratorio agudo grave (SARS-CoV-2). Al inicio surgieron muchas dudas relacionadas con los ajustes al tratamiento y el seguimiento de los pacientes con leucemias crónicas y diversas asociaciones internacionales emitieron recomendaciones. El uso de la telemedicina y la selección de estrategias de tratamiento que permitan un menor acercamiento de los pacientes a los centros hospitalarios ha sido una de las principales técnicas de protección de los pacientes con leucemias crónicas. La experiencia internacional nos describe que al parecer los pacientes con leucemia mieloide crónica son menos susceptibles a contraer la infección y a morir, al contrario de los pacientes con leucemia linfocítica crónica.

Abstract On January 30 of the 2020 year, the World Health Organization declared an international public health emergency the pandemic caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Initially, many doubts arose regarding treatment adjustments and follow-up of patients with chronic leukemias, and various international associations issued recommendations. The use of telemedicine and the selection of treatment strategies that allow care without patients going to hospital centers, has been one of the main techniques for protecting patients with chronic leukemias. International experience describes that it seems that patients with chronic myeloid leukemia are less susceptible to infection and death, unlike patients with chronic lymphocytic leukemia.

Concordancia diagnóstica del sistema de graduación Chisholm Mason y Focus Score en la sialoadenitis linfocítica focal como aproximación al síndrome de Sjögren / Diagnostic agreement of the Chisholm Mason and Focus Score grading system in focal lymphocytic sialadenitis in patients with dry symptoms

RESUMEN Introducción: Existen diferentes sistemas de graduación para interpretar la biopsia de glándula salival menor (BGSM) en pacientes con síntomas secos. En la actualidad la metodología recomendada (Focus Score [FS]), aunque no se emplea de forma rutinaria, conlleva discrepancias diagnósticas. Objetivo: Determinar la concordancia de la lectura histopatológica de BGSM entre las técnicas FS y Chisholm Mason (CM) para el diagnóstico de sialoadenitis linfocítica focal en el hospital de San José, entre enero de 2017 y junio de 2018. Métodos: Estudio de concordancia. Se incluyeron BGSM de pacientes con síntomas secos. Se excluyó material no apto para el estudio, o con menos de 4 lóbulos o menos de 8 mm de área. Se clasificaron utilizando las técnicas FS y CM por 2 evaluadores independientes. Se evaluó el acuerdo interobservador, intraobsevador y global entre las pruebas mediante el coeficiente de Kappa de Cohen. Resultados: Se evaluaron 130 biopsias. Los valores K intraobservador entre FS y CM fueron 0,42 para el observador 1 y 0,23 para el observador 2. Los valores K del acuerdo interobservador fueron 0,47 para FS y 0,65 para CM. La concordancia entre los 2 sistemas de puntuación fue K de 0,13. Conclusiones: Se evidencia una probable sobreestimación de focos con CM, que resalta sus debilidades, y mayor precisión con FS. Por ende, la concordancia entre las 2 pruebas es débil. Es necesario un mayor esfuerzo para el uso del método estandarizado de lectura de BGSM con el FS para una correcta interpretación y beneficio en la clasificación de pacientes con síndrome seco.

ABSTRACT Introduction: There are different grading systems for interpreting the minor salivary gland biopsy (MSGB) in patients with dry symptoms (Sicca). The recommended methodology is currently the Focus Score (FS), which, although not performed routinely, results in diagnostic discrepancies. Objective: To determine the concordance of the histopathological reading of MSGB between the FS and Chisholm Mason (CM) techniques for diagnosis of focal lymphocytic sialadenitis at the San José Hospital between January 2017 and June 2018. Methods: Concordance study. MSGB of patients with sicca symptoms were included. Material not suitable for study and / or with less than 4 lobules and / or less than 8 mm of surface area was excluded. They were classified, using the FS and CM techniques, by two independent evaluators. The inter-observer, intra-observer, and overall agreement between the tests were evaluated using Cohen's Kappa coefficient. Results: A total of 130 biopsies were evaluated. The intra-observer K values between FS and CM were 0.42 for observer 1, and 0.23 for observer 2. The K values of the inter-observer agreement were 0.47 for FS, and 0.65 for CM. The concordance between the two scoring systems was K = 0.13. Conclusions: There is evidence of a probable over-estimation of foci with CM, highlighting its weaknesses and greater precision with FS technique, resulting in the weak concordance between the two tests. A greater effort is necessary for the use of the standardised MSGB FS reading method for a correct interpretation and benefit in the classification of patients with dry syndrome.

Neumonía intersticial linfocítica en una paciente con lupus eritematoso sistémico y síndrome de Sjögren secundario: reporte de caso y revisión de la literatura / Lymphocytic interstitial pneumonia in a patient with systemic lupus erythematosus and secondary Sjögren's syndrome: Case report and literature review

RESUMEN La neumonía intersticial linfocítica es una complicación, poco frecuente, asociada con el lupus eritematoso sistémico, sin embargo, con gran impacto en la calidad de vida. Se asocia con la presencia de anti Ro/SSA, anti La/SSB y con el diagnóstico de síndrome de Sjögren secundario. No es clara la estrategia terapéutica y la información existente está basada en reportes de caso sin disponibilidad de estudios adecuadamente diseñados. En el presente documento se expone el caso de una paciente con lupus eritematoso sistémico y síndrome de Sjögren secundario, que presentó una enfermedad pulmonar intersticial con características de neumonía intersticial linfocítica.

ABSTRACT Lymphocytic interstitial pneumonia is an infrequent complication associated with systemic lupus erythematosus and has a great impact on quality of life. It is associated with the presence of anti-Ro/SSA, anti-La/SSB, and the diagnosis of secondary Sjögren Syndrome. Its therapeutic strategy is not clear, and the existing information is based on case reports, with there being no properly designed studies available. The case is presented here of a patient with systemic lupus erythematosus and secondary Sjögren's syndrome, who also presented with interstitial lung disease with characteristics of lymphocytic interstitial pneumonia.

Lymphocytic interstitial pneumonia: computed tomography findings in 36 patients / Pneumonia intersticial linfocítica: achados na tomografia computadorizada em 36 pacientes

Abstract Objective: To analyze the computed tomography (CT) findings of lymphocytic interstitial pneumonia (LIP). Materials and Methods: We retrospectively reviewed the clinical and CT findings of 36 patients with LIP, including 25 women and 11 men, with a mean age of 52.5 years (age range, 22-78 years). Results: The main associated diseases with LIP were Sjögren syndrome (42%), human immunodeficiency virus infection (17%), amyloidosis (17%), Sjögren syndrome associated with secondary amyloidosis (11%), idiopathic (8%), and systemic lupus erythematosus (5%). The predominant CT abnormalities were multiple cystic airspaces (n = 35), small nodules (n = 15), ground-glass opacities (n = 13), bronchiectasis and/or bronchiolectasis (n = 8), and thickening of the bronchovascular bundles (n = 8). Other CT findings included reticular opacities (n = 7), calcified nodules (n = 4), airspace consolidation (n = 4), emphysema (n = 3), honeycombing (n = 3), lymph node enlargement (n = 2), mosaic attenuation pattern (n = 1), and cavitated nodules (n = 1). Conclusion: The main CT findings of LIP were multiple cysts, small nodules, and ground-glass opacities.

Resumo Objetivo: Analisar os achados da tomografia computadorizada da pneumonia intersticial linfocítica. Materiais e Métodos: Trinta e seis pacientes com diagnóstico de pneumonia intersticial linfocítica (25 mulheres, 11 homens; faixa etária de 22 a 78 anos; idade média de 52,5 anos) tiveram os achados clínicos e tomográficos revisados retrospectivamente. Resultados: As principais doenças associadas à pneumonia intersticial linfocítica foram síndrome de Sjögren (42%), infecção pelo HIV (17%), amiloidose (17%), síndrome de Sjögren associada a amiloidose secundária (11%), síndrome de Sjögren idiopática (8%) e lúpus eritematoso sistêmico (5%). As alterações predominantes na TC consistiram em múltiplos espaços aéreos císticos (n = 35), pequenos nódulos (n = 15), opacidades em vidro fosco (n = 13), bronquiectasias e bronquiolectasias (n = 8) e espessamento de feixes broncovasculares (n = 8). Outros achados da TC incluíram opacidades reticulares (n = 7), nódulos calcificados (n = 4), consolidação do espaço aéreo (n = 4), enfisema (n = 3), faveolamento (n = 3), linfonodomegalia linfática (n = 2), padrão de atenuação em mosaico (n = 1) e nódulos escavados (n = 1). Conclusão: Os principais achados tomográficos observados em pacientes com pneumonia intersticial linfocítica foram múltiplos cistos, pequenos nódulos e opacidades em vidro fosco.

Linfoma linfocítico de células pequeñas de anexos oculares. Presentación de un caso / Small cell lymphocytic lymphoma of eye annexes. Case presentation

RESUMEN Los linfomas son enfermedades malignas que se originan en las células del sistema inmune y se manifiestan predominantemente por linfoadenopatías; en ocasiones la presentación es en sitios extranodales. El ojo y sus anexos pueden ser afectados por un grupo heterogéneo de linfomas primarios o, secundariamente, por la extensión de linfomas originados en ganglios linfáticos o en otros sitios extranodales. Se reporta el caso de un paciente que acudió a consulta por ptosis palpebral izquierda y al examen físico se detectó afectación bilateral, con presencia de tumores en la conjuntiva bulbar inferior en ojo derecho y en la conjuntiva bulbar superior en ojo izquierdo. Mediante biopsia de conjuntiva se diagnosticó linfoma no Hodgkin linfocítico de células pequeñas B. El paciente fue considerado un estadio IIIAE de Ann Arbor y tratado con seis ciclos de ciclofosfamida, vincristina, doxorrubicina y prednisona, más el anticuerpo monoclonal cubano anti-CD20 CIMABior®. Se alcanzó la respuesta completa, estado en el cual se mantiene hasta el momento.

ABSTRACT Lymphomas are malignant diseases that originate in the immune system cells and are predominantly manifested by lymphadenopathy; sometimes the presentation is in extranodal places. The eye and its annexes may be affected by a heterogeneous group of primary lymphomas or, secondarily, by the extension of lymphomas originated in lymph nodes or other extranodal sites. The case of a patient who went to the consultation due to left palpebral ptosis is reported and physical examination showed bilateral involvement, with tumors in the lower right eye bulbar conjunctiva and in the left eye upper bulbar conjunctiva. A small B cell lymphocytic non-Hodgkin lymphoma was diagnosed by conjunctiva biopsy. The patient was considered a stage IIIAE of Ann Arbor and treated with six cycles of cyclophosphamide, vincristine, doxorubicin and prednisone, plus the Cuban monoclonal antibody anti-CD20 CIMABior®. The complete response was reached, state in which the disease remains so far.