Linfoma anaplásico de grandes células T associado ao uso de implantes (BIA-ALCL): relato de caso / Implant-associated anaplastic large T-cell lymphoma (BIA-ALCL): case report

Reconhecido pela Organização Mundial de Saúde em 2016, o linfoma anaplásico de grandes células associado ao implante mamário (BIA-ALCL) é um subtipo incomum de linfoma não Hodgkin de células T, que se desenvolve após a inserção de próteses mamárias. A doença é uma afecção rara que afeta cerca de uma a cada 30.000 pessoas com implante mamário texturizado. As principais manifestações clínicas são o seroma tardio, assimetria mamária, massa e contratura capsular, com frequência mais elevada do primeiro. O explante da prótese com capsulectomia total pode ser suficiente para tratar o ALCL, com ressecções estendidas a locais adjacentes, quando necessário. Entretanto, em alguns casos, é realizada a radioterapia e/ou quimioterapia adjuvante. Conclui-se que, para um diagnóstico precoce e um tratamento efetivo, mulheres com seroma de aparecimento súbito e tardio deverão realizar exames complementares para a exclusão dessa afecção, mesmo com tempo inferior à média de desenvolvimento, que é de cerca de 10,6 anos.

Recognized by the World Health Organization in 2016, breast implant-associated anaplastic large cell lymphoma (BIA-ALCL) is an uncommon subtype of T-cell non-Hodgkin lymphoma that develops after the insertion of breast implants. The disease is a rare condition that affects approximately one in every 30,000 people with textured breast implants. The main clinical manifestations are late seroma, breast asymmetry, mass, and capsular contracture, with a higher frequency of the former. Explantation of the prosthesis with total capsulectomy may be sufficient to treat ALCL, with resections extended to adjacent sites when necessary. However, in some cases, adjuvant radiotherapy and/or chemotherapy is performed. It is concluded that, for an early diagnosis and effective treatment, women with sudden and late-onset seroma should undergo additional tests to exclude this condition, even with a shorter development time than the average, which is around 10.6 years.

Masa paravertebral torácica como manifestación infrecuente del Linfoma de alto grado: Reporte de un caso

Paciente masculino de 44 años sin antecedentes patológicos previos, acude por cuadro de disnea y dolor pleurítico en hemitórax derecho. La radiografía de tórax tomada a su ingreso muestra importante derrame pleural derecho. Fue evaluado por el servicio de Neumología quienes realizan toracocentesis diagnóstica y evacuadora. Además, solicitaron tomografía contrastada de tórax, la cual reporta una gran masa mediastínica posterior (paravertebral), asociada a aparente infiltración de la médula ósea en múltiples cuerpos vertebrales, y adenopatías inguinales bilaterales. Posteriormente se realiza biopsia de la masa paravertebral, confirmando uno de los diagnósticos infrecuentes presentados como masas del mediastino posterior, Linfoma de células B grande. (provisto por Infomedic International)

A 44-year-old male patient with no previous medical history presented with dyspnea and pleuritic chest pain in the right hemithorax. The chest x-ray taken upon admission shows significant right pleural effusion. He was evaluated by the Pulmonology department who performed diagnostic and evacuative thoracentesis. In addition, they requested contrast-enhanced chest tomography, which reported a large posterior mediastinal (paravertebral) mass, associated with apparent bone marrow infiltration of multiple vertebral bodies, and bilateral inguinal lymphadenopathy. Subsequently, a biopsy of the paravertebral mass was performed, confirming one of the rare diagnoses presented as posterior mediastinal masses, large B-cell Lymphoma. (provided by Infomedic International)

Linfoma de células B primario de mama en una paciente con sida. Presentación de un caso y revisión de la literatura / Primary B-cell lymphoma of the breast in an AIDS patient. Case report and literature review

Los LNH constituyen la segunda neoplasia más frecuente en pacientes con VIH. Estas neoplasias están ligadas a la inmunodeficiencia, suelen ser de período de latencia prolongado y más frecuentes en hombres. Más del 95% de estas neoplasias son de fenotipo B, de alto grado de malignidad, extranodales y representan la causa de muerte en un 12% al 16% de los casos. El linfoma no Hodgkin primitivo de mama (LPM) es una entidad infrecuente, que representa el 2,2% de todos los linfomas extranodales y el 0,5% de todas las neoplasias malignas de la mama. Se presenta una mujer con sida y linfoma primario de mama. (AU)

NHL is the second most common neoplasm in patients with HIV. It is linked to immunodeficiency, tends to have a long latency period and is more common in men. More than 95% of these neoplasms are of phenotype B, high-grade, extranodal and are the cause of death in 12% to 16% of cases. Primitive non-Hodgkin lymphoma of the breast is a rare entity, accounting for 2.2% of all extranodal lymphomas and 0.5% of all breast malignancies. A woman with AIDS and primary breast lymphoma is presented. (AU)

Linfoma extranodal de epiglotis en un paciente con recaída de leucemia linfoide aguda de células T. Presentación de un caso / Extranodal lymphoma of the epiglottis in a patient with relapsed T-cell acute lymphoid leukemia. Case report

Los linfomas localizados en la laringe representan un porcentaje muy bajo dentro de los comprendidos en los tumores de cabeza y cuello en la edad pediátrica. El linfoma no Hodgkin es el subtipo más comúnmente reportado en la literatura, el cual dependiendo de su etiología y extensión determinará el pronóstico del paciente. La certeza del diagnóstico, que suele ser muy difícil de alcanzar, se confirma generalmente mediante una biopsia de tejido. En la actualidad, no hay reportes de la literatura acerca de linfomas leucemoides diseminados a laringe. Se presenta el caso de un paciente masculino adolescente de 17 años con diagnóstico de una leucemia linfoide aguda con recaída extra-nodal en la laringe por falla en el esquema quimioterapéutico instaurado.

Lymphomas located at the level of the larynx represent a very low percentage of head and neck tumors in the pediatric age group. Non-Hodgkin's lymphoma is the most reported subtype in the literature, which depending on its etiology and extension will determine the patient's prognosis. Diagnostic certainty, which is often very difficult to achieve, is usually confirmed by tissue biopsy. At present, there are no reports in the literature about leukemoid lymphomas disseminated to the larynx. We present the case of a 17-year-old adolescent male patient diagnosed with acute lymphoid leukemia with extranodal relapse in the larynx due to failure of the chemotherapeutic regimen.

Enfermedad de Castleman: un enfoque integral a propósito de un reporte de caso / Castleman's Disease: A Comprehensive Approach Regarding a Case Report

Resumen Se presenta el caso clínico de una paciente del sexo femenino, de 30 años, con adenopatías supraclaviculares y axilares ipsilaterales, quien fue sometida a biopsia excisional con posterior estudio. El análisis histopatológico de la muestra de tejido resecado reveló una serie de características distintivas asociadas con la enfermedad de Castleman variante hialinovascular. La presentación de este caso no solo proporciona información detallada sobre la evolución clínica de la paciente, sino que también sirve como base para ilustrar los aspectos clave del diagnóstico histopatológico y las implicaciones inmunohistoquímicas en la enfermedad de Castleman. Además de hacer una revisión de tema respecto a esta patología poco común, en la cual los informes de casos son fundamentales para aumentar la comprensión de su variabilidad clínica y su abordaje diagnóstico, ilustrando los desafíos en el diagnóstico diferencial y como deben abordarse los mismos.

Abstract The clinical case of a 30-year-old female patient with supraclavicular and ipsilateral axillary lymphadenopathy who underwent excisional biopsy with subsequent study is presented. Histopathological analysis of the resected tissue sample revealed a series of distinctive features associated with hyalinevascular variant Castleman disease. The presentation of this case not only provides detailed information about the clinical evolution of the patient, but also serves as a basis to illustrate Key aspects of histopathological diagnosis and immunohistochemical implications in Castleman disease. In addition to making a review of the topic regarding this rare pathology in which case reports are essential to increase the understanding of its clinical variability and its diagnostic approach, illustrating the challenges in differential diagnosis and how they should be addressed.

Evolução da Captação Miocárdica de 18F-FDG em Paciente com Diagnóstico de Cardiotoxicidade / Progression of Myocardial 18F-FDG Uptake in a Patient with Cardiotoxicity

Resumo O objetivo deste relato é mostrar a evolução da cardiotoxicidade (CTX) por quimioterápicos em paciente com linfoma por exames de imagens, destacando a importância da captação miocárdica de flúor-18 fluordeoxiglicose (18F-FDG) pela tomografia por emissão de pósitrons, acoplada à tomografia computadorizada (PET/CT). Feminino, 43 anos, com linfoma uterino, submetida a histerectomia, três esquemas de quimioterapia (QT), sucessivamente, e radioterapia. Apresentou episódios de insuficiência cardíaca aguda dois anos após QT. Ecocardiograma mostrou redução da fração de ejeção do ventrículo esquerdo (FEVE). Análise retrospectiva do 18F-FDG PET/CT observou elevação da captação miocárdica em todos os exames durante o seguimento oncológico. Apesar da remissão oncológica, a paciente desenvolveu IC com FEVE reduzida. Durante a QT, ocorreu aumento difuso e significativo da captação miocárdica de 18F-FDG, que precedeu a queda do desempenho cardíaco, e pareceu refletir alterações metabólicas nos cardiomiócitos relacionadas à CTX. A análise da captação miocárdica de 18F-FDG modificaria o desfecho cardiológico da paciente? Esse questionamento é relevante, visto que outros pacientes podem se beneficiar desse método como marcador precoce de CTX. Os exames de imagem são imprescindíveis no acompanhamento de pacientes com risco de CTX. O ecocardiograma permanece como principal auxílio diagnóstico, porém o 18F-FDG PET/CT pode estar surgindo como uma poderosa ferramenta para um diagnóstico mais precoce dessa condição clínica.

Abstract The objective of this case report was to present the progression of chemotherapy-induced cardiotoxicity in a patient with lymphoma, highlighting the importance of myocardial fluor-18-fluorodeoxyglucose (18F-FDG) uptake by positron emission tomography coupled with computed tomography (PET/CT). 43-year-old female patient with uterine lymphoma, who underwent hysterectomy followed by three chemotherapy regimens and radiotherapy. The patient had episodes of acute heart failure two years after chemotherapy. Echocardiogram revealed a reduction in left ventricular ejection fraction (LVEF). A retrospective analysis of 18F-FDG PET/CT showed an increase in myocardial uptake in all tests performed during oncologic treatment. Despite disease remission, the patient developed heart failure with reduced LVEF. During chemotherapy, there was a diffuse, significant increase in myocardial 18F-FDG uptake, which preceded the decrease in myocardial performance and seemed to reflect metabolic changes in cardiomyocytes, related to cardiotoxicity. Would an analysis of myocardial 18F-FDG uptake yield a different cardiac outcome in this patient? This question is relevant, considering that other patients may benefit from the use of PET as an early marker of cardiotoxicity. Imaging tests are essential in the follow-up of patients at risk of cardiotoxicity. Although echocardiography remains the main imaging test in the diagnosis of cardiotoxicity, 18F-FDG PET/CT may be a powerful tool for the early diagnosis of this condition.

Risco de recorrência em Sistema Nervoso Central de Linfoma Difuso de grandes células B e sua profilaxia em Serviço Público de Saúde em dois centros no Brasil / Risk of recurrence in the Central Nervous System of Diffuse Large B-Cell Lymphoma and its prophylaxis in a Public Health Service in two centers in Brazil

O linfoma difuso de grandes células B (LDGCB) é o subtipo mais comum de linfoma não Hodgkin. A recaída em sistema nervoso central (SNC) é um evento raro, variando de 5% a 10%, de acordo com fatores de risco previamente definidos através do Índice Prognóstico Internacional do SNC (CNS-IPI) e sítios extranodais específicos. Apresenta desfechos insatisfatórios, com sobrevida global mediana de dois a cinco meses. Ao longo dos anos, diversas estratégias para reduzir a recaída em SNC foram avaliadas, e são cada vez mais controversas. As profilaxias para evitar recaída em SNC frequentemente utilizadas diferem na forma de administração, baseados em metotrexato intratecal (IT-MTX) ou de forma sistêmica em altas doses (HD-MTX), associado ou não a outros agentes quimioterápicos. Os estudos até então disponíveis foram realizados em países de alta renda e é questionado se limitações encontradas em países de transição econômica, com maior dificuldade de acesso a métodos diagnósticos e terapêuticos, trariam impacto ou poderiam justificar profilaxia para recaída em SNC. Realizamos um estudo retrospectivo em dois centros de saúde pública em Belo Horizonte, Brasil, entre janeiro de 2018 e julho de 2022, para avaliar a incidência de recaída em SNC em pacientes acometidos por LDGCB. Estimamos sobrevida livre de progressão e sobrevida global. Um total de 120 pacientes, com idade média de 54,4 ± 15,4 anos e predomínio do sexo masculino (60,0%) foram avaliados no estudo. Destes, apenas sete (5,8%) receberam IT-MTX e quatro (3,3%) receberam HD-MTX. Não houve pacientes que receberam as duas vias de profilaxia. O escore prognóstico para risco inicial de recaída do SNC pelo CNS-IPI foi estimado como: baixo [0-1; 37 (30,8%)], moderado [2-3; 53 (44,2%)] ou alto [≥ 4; 27 (22,5%)]. A recaída em SNC foi confirmada em quatro (3,3%) pacientes. Apesar do estudo ter sido realizado em centros de referência oncohematológicas, o n disponível foi pequeno ao considerar a raridade do evento. Não conseguimos demonstrar se há benefício ou não de profilaxia específica para recaída em SNC. Considerando a morbimortalidade desta complicação, sugere-se realizar mais estudos e investigar acometimento oculto de SNC em LDGCB ao diagnóstico.

Diffuse large B-cell lymphoma (DLBCL) is the most common subtype of non-Hodgkin lymphoma. Central nervous system (CNS) relapse is a rare event, varying from 5% to 10%, according to risk factors previously defined through the CNS International Prognostic Index(CNS-IPI) and specific extranodal sites. It presents unsatisfactory outcomes, with a median overall survival of two to five months. Over the years, several strategies to reduce CNS relapse have been evaluated, and they are increasingly controversial. Prophylaxis to prevent CNS relapse frequently used differs in the form of administration, based on intrathecal methotrexate (IT-MTX) or high-dose systemic (HD-MTX), associated or not with other chemotherapeutic agents. The studies available so far were carried out in high-income countries and it is questioned whether limitations found in economic transition countries, with greater difficulty in accessing diagnostic and therapeutic methods, would have an impact or could justify prophylaxis for CNS relapse. We carried out a retrospective study in two public health centers in Belo Horizonte, Brazil, between January 2018 and July 2022, to evaluate the incidence of CNS relapse in patients affected by DLBCL. We estimated progression-free survival and overall survival. A total of 120 patients, with a mean age of 54.4 ± 15.4 years and a predominance of males (60.0%) were evaluated in the study. Of these, only seven (5.8%) received IT-MTX and four (3.3%) received HD-MTX. There were no patients who received both routes of prophylaxis. The prognostic score for initial risk of CNS relapse by CNS-IPI was estimated as: low [0-1; 37 (30.8%)], moderate [2-3; 53 (44.2%)] or high [≥ 4; 27 (22.5%)]. CNS relapse was confirmed in four (3.3%) patients. Although the study was carried out in oncohematological reference centers, the number available was small considering the rarity of the event. We were unable to demonstrate whether or not there is benefit from specific prophylaxis for CNS relapse. Considering the morbidity and mortality of this complication, it is suggested to carry out further studies and investigate occult CNS involvement in DLBCL at diagnosis.

Avaliação das manifestações clínicas e microscópicas dos linfomas em glândulas salivares maiores / Assessment of clinical and microscopic manifestations of lymphomas in major salivary glands

Os subtipos de linfomas não Hodgkin representam 2,8% de todos os novos casos de câncer no mundo, sendo o terceiro grupo mais comum de neoplasias malignas da região de cabeça e pescoço. As glândulas salivares maiores representam o terceiro sítio extranodal mais acometido pelo linfoma na região da cabeça e pescoço; entretanto, nas glândulas salivares maiores é muito raro, representando aproximadamente 1,7­3,1% de todas as neoplasias das glândulas salivares, acometendo a maioria dos casos as glândulas parótidas (79%), seguidas pelas glândulas submandibulares (18%) e sublinguais (1%). Os subtipos mais comuns são linfoma do tecido linfoide associado à mucosa (MALT), o linfoma folicular (FL) e o linfoma difuso de grandes células B (DLBCL), e a frequência destas neoplasias está associado com a ocorrência simultânea de condições sistêmicas que predispõem ao desenvolvimento de neoplasias linfoides como a Síndrome de Sjögren (SS). Entretanto, a literatura sobre linfomas em glândulas maiores permanece muito escassa e impede que conheçamos de forma apropriada as características destes pacientes. Assim, o objetivo deste estudo é avaliar as manifestações clínicas e microscópicas dos linfomas em glândulas salivares maiores. Para isto, foram recuperados de forma retrospectiva dos arquivos de patologia de algumas instituições todos os casos diagnosticados como linfomas acometendo estes sítios anatômicos. Foram coletados os dados clínicos referentes ao sexo, idade, localização, apresentação clínica, tempo de evolução, status, estadiamento e ocorrência da SS, e as informações histopalógicas foram coletadas de blocos de parafina e lâminas em hematoxilina e eosina e imuno-histoquímicas acessíveis. Os resultados obtidos foram avaliados de forma descritiva. As séries compreenderam de 7 casos de linfomas em glândula sublingual, 16 casos em glândula submandibular e 12 casos em glândula parótida. Clinicamente, os linfomas apresentam-se como aumento de volume assintomático, sendo os subtipos mais frequentes os de células B maduras de baixo grau (MALT, FL, MCL), mas subtipos de alto grau também foram observados (LDGCB, SOE). Dois pacientes, um de linfoma de células do manto (LCM) e outro de LDGCB,SOE em glândula sublingual apresentaram como doença disseminada, e apenas três casos de linfoma MALT em glândula parótida apresentam a SS. O tratamento dependeu do microscópico e estágio do tumor, variando de cirurgia, regimes quimioterápicos com R-CHOP e radioterapia. O prognóstico foi favorável principalmente para os casos de baixo grau (MALT,FL), e apenas dois pacientes de sublingual (LDGCB,SOE, MCL) e três de submandibular (LDGCB,SOE, linfoma plasmablastico e MALT) faleceram após o diagnóstico. Neste estudo concluímos que os linfomas em glândulas salivares maiores são afetados principalmente por neoplasias de células B maduras de baixo grau (MALT, FL, MCL) e esses pacientes devem passar por uma avaliação sistêmica criteriosa para determinar se a doença se trata de uma neoplasia primária ou disseminada.

Non-Hodgkin's lymphomas account for 2.8% of all new cancer cases worldwide and are the third most common group of malignant neoplasms in the head and neck region. The major salivary glands represent the third most common extranodal site affected by lymphoma in the head and neck region; however, in the major salivary glands it is very rare, representing approximately 1.7-3.1% of all salivary gland neoplasms, affecting most cases in the parotid glands (79%), followed by the submandibular glands (18%) and sublingual glands (1%). The most common subtypes are mucosa- associated lymphoid tissue lymphoma (MALT), follicular lymphoma (FL) and diffuse large B-cell lymphoma (DLBCL), and the frequency of these neoplasms is associated with the simultaneous occurrence of systemic conditions that predispose to the development of lymphoid neoplasms such as Sjögren's Syndrome(SS). However, the literature on lymphomas in major glands remains very scarce and prevents us from properly understanding the characteristics of these patients. Therefore, the aim of this study was to evaluate the clinical and microscopic manifestations of lymphomas in the major salivary glands. To this end, all cases diagnosed as lymphomas affecting these anatomical sites were retrospectively retrieved from the pathology archives of a number of institutions. Clinical data was collected on gender, age, location, clinical presentation, time of evolution, status, staging and occurrence of SS, and histopathological information was collected from paraffin blocks and slides in hematoxylin and eosin and accessible immunohistochemistry. The results obtained were evaluated descriptively. The series comprised 7 cases of lymphomas in the sublingual gland, 16 cases in the submandibular gland and 12 cases in the parotid gland. Clinically, the lymphomas presented as asymptomatic enlargement, with the most frequent subtypes being low-grade mature B-cells (MALT, FL, MCL), but high- grade subtypes were also observed (LDGCB, SOE). Two patients, one with mantle cell lymphoma (MCL) and the other with LDGCB,SOE in the sublingual gland presented with disseminated disease, and only three cases of MALT lymphoma in the parotid gland presented with SS. Treatment depended on the microscopic subtype and stage of the tumor, ranging from surgery to chemotherapy regimens with R-CHOP and radiotherapy. Prognosis was mainly favorable for low-grade cases (MALT,FL), and only two sublingual patients (LDGCB,SOE, MCL) and three submandibular patients (LDGCB,SOE, plasmablastic lymphoma and MALT) died after diagnosis. In this study we conclude that lymphomas in the major salivary glands are mainly affected by low- grade mature B-cell neoplasms (MALT, FL, MCL) and these patients should undergo a careful systemic evaluation to determine whether the disease is a primary or disseminated neoplasm.

Lesiones difusas nodulares endobronquiales masivas en paciente con linfoma No Hodgkin / Massive Diffuse Nodular Endobronchial Lesions in a Patient with Non-Hodgkin Lymphoma

RESUMEN El linfoma no Hodgkin compromete en un 50 % de los casos estructuras intratoráci cas; la afectación bronquial es infrecuente. Presentamos el caso de una paciente con sospecha de enfermedad linfoproliferativa e infiltrados pulmonares. En la broncoscopia se observaron imágenes nodulares difusas prominentes de todo el árbol bronquial. La anatomía patológica de la biopsia bronquial fue compatible con linfoma no Hodgkin marginal y la evolución con quimioterapia fue favorable.

ABSTRACT Thoracic involvement occurs in 50 % of cases of Non-Hodgkin lymphoma. Bronchial involvement is rare. We describe a case presenting with probable lymphoproliferative disease and pulmonary infiltrates. Bronchoscopy revealed prominent diffuse nodular images throughout the bronchial tree. Bronchial biopsy yielded a diagnosis of marginal Non-Hodgkin Lymphoma. The patient had a good response to chemotherapy.

Marginal Zone B-cell Lymphoma of the Gasserian Ganglion: Case Report and Review of the Literature / Linfoma de células B da zona marginal do gânglio de Gasser: Relato de caso e revisão da literatura

Primary central nervous system lymphoma (PCNSL) is a rare subtype of extranodal non-Hodgkin's lymphoma that accounts for 4% of newly diagnosed central nervous system (CNS) tumors. Most primary lymphomas of the central nervous system are of the subtype of diffuse large B-cell lymphomas, which have highly aggressive behavior and may involve the brain, leptomeninges, eyes or spinal cord without evidence of systemic disease. Primary CNS lymphomas are very rare in immunocompetent patients, but their rates are increasing. So far, only 11 primary Gasser ganglion lymphomas have been reported, with an incidence of 2.5 cases per 30,000,000 inhabitants. However, B cell lymphomas of the marginal zone of the Gasserian ganglion have been very rarely reported. We report here a clinical presentation characteristic of B cell lymphoma of the marginal zone of the Gasser ganglion in an immunocompetent patient who was treated with surgery and radiotherapy, evolving with improvement of symptoms and without recurrence in 3 months of follow-up.

O linfoma primário do sistema nervoso central (PCNSL, na sigla em inglês) é um subtipo raro de linfoma não-Hodgkin extranodal que representa 4% dos tumores recém-diagnosticados do sistema nervoso central (SNC). A maioria dos linfomas primários do sistema nervoso central é do subtipo dos linfomas difusos de grandes células B, que tem comportamento altamente agressivo e pode envolver cérebro, as leptomeninges, os olhos ou a medula espinhal sem evidências de doença sistêmica. Os linfomas primários do SNC são muito raros em pacientes imunocompetentes, mas suas taxas estão aumentando. Até o momento, apenas 11 linfomas primários do gânglio de Gasser foram relatados, com uma incidência de 2,5 casos por 30.000.000 de habitantes. No entanto, linfomas de células B da zona marginal do gânglio de Gasserian foram muito raramente relatados. Relatamos aqui uma apresentação clínica característica de um linfoma de células B da zona marginal do gânglio de Gasser em um paciente imunocompetente que foi tratado com cirurgia e radioterapia, evoluindo com melhora dos sintomas e sem recorrência em três meses de acompanhamento.

Linfadenopatías generalizadas como manifestación inicial de lupus eritematoso sistémico: a propósito de un caso clínico

La presencia de linfadenopatías generalizadas se ha asociado de forma usual con lupus eritematoso sistémico (LES), sin embargo, no es usual encontrar este hallazgo como manifestación inicial de la enfermedad. Existen múltiples diagnósticos diferenciales que incluyen la linfadenitis necrotizante histiocítica de Kikuchi, la enfermedad de Castleman, infecciones y el linfoma cuando se presenta este hallazgo como síntoma inicial de LES. Presentamos el caso de un hombre de 56 años que se presentó con 2 meses de linfadenopatía generalizada sin datos al examen o antecedentes que sugirieran diagnóstico de LES; se sospechó inicialmente linfoma o enfermedad infecciosa y se realizó un estudio exhaustivo incluido biopsia de ganglio cervical. La investigación de laboratorio finalmente reveló leucopenia, proteinuria significativa, ANA y anti-dsDNA positivos e hipocomplementemia, lo que confirma el diagnóstico de enfermedad autoinmune tipo LES. Este caso ilustra la importancia de reconocer esta forma de presentación inusual, dado que se trata de una enfermedad potencialmente fatal.

The presence of generalized lymphadenopathy has usually been associated with systemic lupus erythematosus (SLE), however, it is not usual to find this finding as an initial manifestation of the disease. There are multiple differential diagnoses that include Kikuchi histiocytic necrotizing lymphadenitis, Castleman disease, infections and lymphoma when this finding is presented as an initial symptom of SLE. We present the case of a 56-year-old man who presented with 2 months of generalized lymphadenopathy without examination findings or history suggesting a diagnosis of SLE; Lymphoma or infectious disease was initially suspected and an exhaustive study was performed, including cervical lymph node biopsy. Laboratory investigation finally revealed leukopenia, significant proteinuria, positive ANA, positive anti-dsDNA, and hypocomplementemia, confirming the diagnosis of SLE-type autoimmune disease. This case illustrates the importance of recognizing this unusual presentation, given that it is a potentially fatal disease.

Síndrome de dificultad respiratoria aguda por Mycobacterium tuberculosis de rápido crecimiento en una mujer con virus linfotrópico T humano tipo I-II / Acute respiratory distress syndrome due to rapidly growing Mycobacterium tuberculosis in a woman with human T-lymphotropic virus type I-II

La tuberculosis es una infección de distribución mundial con una alta morbimortalidad en países subdesarrollados. Presentamos el caso de una mujer de 55 años con cuadro de dos meses de adenomegalias cervicales, pérdida de peso y fiebre con posterior disnea de esfuerzo progresiva. Ingresó por urgencias en falla ventilatoria y hallazgos radiológicos compatibles con síndrome de dificultad respiratoria aguda (SDRA) requiriendo ventilación mecánica invasiva. Se demostró infección por M. tuberculosis por PCR en tiempo real GeneXpert MTB/RIF Ultra y en cultivo de medio líquido de esputo, con crecimiento en tiempo menor a tres días. La biopsia de las adenomegalias confirmó linfoma de células T periférico asociado a infección por virus linfotrópico T humano tipo I-II, presentando una evolución tórpida a pesar del esquema quimioterapéutico.

Tuberculosis is a globally distributed infection with high morbidity and mortality in underdeveloped countries. We present the case of a 55-year-old woman with a 2-month history of cervical lymph nodes, weight loss, and fever with subsequent progressive exertional dyspnea. She was admitted to the emergency department with ventilatory failure and radiological findings compatible with acute respiratory distress syndrome (ARDS) requiring invasive mechanical ventilation. M. tuberculosis infection was proved by a real-time PCR GeneXpert MTB/RIF Ultra and in culture of sputum liquid medium, with growth in less than 3 days. The biopsy of the lymph nodes revealed peripheral T-cell lymphoma associated with infection by human T-lymphotropic virus type I-II, presenting a torpid evolution despite the chemotherapy regimen.

Causa inusual de obstrucción traqueal: linfoma de células B no Hodgkin / Unusual cause of tracheal obstruction: B cell non-Hodgkin lymphoma

Las neoplasias de la tráquea son poco comunes, siendo más infrecuente aún el linfoma de células B no Hodgkin a este nivel. La presentación clínica de esta entidad es muy variable y sus síntomas son muy inespecíficos, simulando varias enfermedades, generando así un diagnóstico tardío. Actualmente se dispone de varias ayudas diagnósticas que permiten descartar otras patologías y confirmar la neoplasia traqueal, además la biopsia es fundamental para llegar al diagnóstico final y así instaurar el manejo en estadios tempranos, para impactar en la morbimortalidad. A continuación presentamos el caso de una paciente con linfoma de células B no Hodgkin como causa de obstrucción traqueal.

Tracheal neoplasms are uncommon, with non-Hodgkin B-cell lymphoma being even rarer at this level. The clinical presentation of this entity is highly variable and its symptoms are very non-specific, simulating several diseases, thus generating a late diagnosis. Currently, there are several diagnostic aids that allow us to rule out other pathologies and confirm tracheal neoplasia, in addition, biopsy is essential to reach the final diagnosis and thus establish management in early stages to impact morbidity and mortality. We present the case of a patient with non-Hodgkin's B-cell lymphoma as a cause of tracheal obstruction.

Adolescence Pseudolymphoma / Pseudolinfoma na adolescência

Objective: report an uncommon case of cutaneous pseudolymphoma in teenage years, undiagnosed for approximately 8 years old. Methodology: data were taken from medical records, patient interviews, photographic records of the injuries, diagnostic methods, and literature review. The paper was subjected and approved by the Research Ethics Committee (REC), under the number 4.952.193, authorized by the patient and their legal sponsor. Final Conclusions: the related case shows the importance of reliable and differential diagnoses since the patient carried the injury through approximately eight years without getting any diagnosis and/or treatment. Furthermore, the unusual age and the location of the injuries make the information presented here fundamental to helping other professionals and contributing to the Public Health System (AU).

Objetivo: Relatar um caso incomum de Pseudolinfoma Cutâneo na adolescência, não diagnosticado por aproximadamente oito anos. Método: As informações foram obtidas pela revisão do prontuário, entrevista com o paciente, registro fotográfico das lesões e dos métodos diagnósticos e revisão de literatura. O trabalho foi submetido e aprovado pelo Comitê de Ética em Pesquisa (CEP), número de aprovação 4.952.193, com autorização do paciente e seus responsáveis legais. Considerações Finais: o caso relatado evidencia a importância de um diagnóstico fidedigno e dos diagnósticos diferenciais, uma vez que a paciente apresentou a lesão por aproximadamente oito anos, sem receber nenhum diagnóstico e/ou tratamento. Além disso, não só a faixa etária é incomum, mas também a localização da lesão e por esta razão, as informações são fundamentais para auxiliar outros profissionais, com benefício deste estudo para o Sistema de Saúde (AU).

Inusual caso de Linfoma B Difuso de Células Grandes (NOS) Primario Cerebral asociado a hidrocefalia y manejo neuroquirúrgico / Unusual case of diffuse B lymphoma of large cells (NOS) primary cerebral associated with hydrocephaly and neurosurgical management

RESUMEN El linfoma cerebral primario es una variedad de linfoma no Hodgkin agresivo que se origina en el sistema nervioso central y se diagnostica cuando no hay evidencia de linfoma en ninguna otra parte del cuerpo. Aunque los linfomas sistémicos son más conocidos y estudiados, el linfoma cerebral primario ha sido menos comprendido debido a su baja frecuencia hasta hace algunas décadas. El tratamiento de esta enfermedad sigue siendo objeto de debate. En esta oportunidad se reporta el inusual caso de linfoma B de células grandes (NOS, del inglés Not Other form Specified); asociada a hidrocefalia y su manejo.

ABSTRACT Primary brain lymphoma is a type of aggressive non-Hodgkin lymphoma that originates in the central nervous system and is diagnosed when there is no evidence of lymphoma anywhere else in the body. Although systemic lymphomas are better known and studied, primary brain lymphoma has been less understood due to its low frequency until a few decades ago. The treatment of this disease continues to be debated. On this occasion, the unusual case of large B cell lymphoma NOS associated with hydrocephalus and its management is reported.

Survival of patients living with HIV and cancer in Cali, Colombia

Background: People living with HIV have an increased risk of cancer compared to the general population. However, with the increase in life expectancy and advances in antiretroviral therapy, the survival of patients with cancer and HIV has changed. Objective: To determine the survival of patients living with HIV and cancer in Cali, Colombia Methods: A retrospective cohort study was conducted at the Fundación Valle del Lili, Cali, Colombia. Data from the HIV database was crossed with data from the hospital and population-based cancer registries between 2011-2019. Patients <18 years, limited available clinical information on the diagnosis and treatment of HIV and cancer, and non-oncological tumor diagnosis were excluded. Results: A total of 173 patients were included. The frequencies of AIDS-defining neoplasms were: Non-Hodgkin lymphoma (42.8%), Kaposi sarcoma (27.8%), and cervical cancer (4.6%). Overall survival was 76.4% (95% CI 68.9-82.3) at five years. Poorer survival was found in patients with AIDS-defining infections (56.9% vs. 77.8%, p=0.027) and non-AIDS-defining infections (57.8% vs. 84.2%, p=0.013), while there was better survival in patients who received antiretroviral therapy (65.9% vs. 17.9%, p=0.021) and oncological treatment (66.7% vs. 35.4%, p<0.001). The presence of non-AIDS-defining infections increases the risk of dying (HR = 2.39, 95% CI 1.05-5.46, p=0.038), while oncological treatment decreases it (HR = 0.33, 95% CI 0.14-0.80, p=0.014). Conclusions: In people living with HIV, Non-Hodgkin lymphoma and Kaposi sarcoma are the most common neoplasms. Factors such as AIDS-associated and non-AIDS-associated infections have been identified as determinants of survival. Cancer treatment seems to improve survival.

Antecedentes: Las personas que viven con VIH tienen un riesgo mayor de cáncer en comparación con la población general. Sin embargo, con el aumento de la esperanza de vida y los avances en la terapia antirretroviral, la supervivencia de los pacientes con cáncer y VIH ha cambiado. Objetivo: Determinar la supervivencia de los pacientes que viven con VIH y cáncer en Cali, Colombia. Métodos: Se realizó un estudio de cohorte retrospectivo en la Fundación Valle del Lili, Cali, Colombia. Los datos de la base de datos de VIH se cruzaron con los datos de los registros de cáncer de base hospitalaria y poblacional entre 2011-2019. Se excluyeron los pacientes <18 años, con información clínica limitada disponible sobre el diagnóstico y tratamiento del VIH y el cáncer y los casos con diagnóstico de tumor no oncológico. Resultados: Se incluyeron un total de 173 pacientes. Las frecuencias de neoplasias definitorias de SIDA fueron: linfoma no Hodgkin (42.8%), sarcoma de Kaposi (27.8%) y cáncer cervical (4.6%). La supervivencia global fue del 76.4% (IC 95% 68.9-82.3) a los cinco años. Se encontró una peor supervivencia en pacientes con infecciones definitorias de SIDA (56.9% vs. 77.8%, p=0.027) e infecciones no definitorias de SIDA (57.8% vs. 84.2%, p=0.013), mientras que hubo una mejor supervivencia en pacientes que recibieron terapia antirretroviral (65.9% vs. 17.9%, p=0.021) y tratamiento oncológico (66.7% vs. 35.4%, p<0.001). La presencia de infecciones no definitorias de SIDA aumentó el riesgo de morir (HR = 2.39, IC 95% 1.05-5.46, p=0.038), mientras que el tratamiento oncológico lo disminuyó (HR = 0.33, IC 95% 0.14-0.80, p=0.014). Conclusiones: En las personas que viven con VIH, el linfoma no Hodgkin y el sarcoma de Kaposi son las neoplasias más comunes. Se han identificado factores como las infecciones asociadas al SIDA y las infecciones no asociadas al SIDA como determinantes de la supervivencia. El tratamiento del cáncer parece mejorar la supervivencia.

COVID-19 y leucemia/linfoma T del adulto, sinergia mortal / COVID-19 and adult T cell leukemia-lymphoma: a deadly synergy

La leucemia/linfoma T del adulto es una neoplasia maligna de mal pronóstico frecuente en población anciana. Se presenta el caso de una mujer de 44 años de edad, de Ayacucho, diagnosticada con el subtipo linfomatoso de esta enfermedad e infección por virus linfotrópico T humano-I; mostró síndrome oclusivo de vena cava superior con tratamiento de quimioterapia sistémica bajo régimen de dosis ajustada con rituximab más etoposido, prednisona, vincristina, ciclofosfamida y doxorubicina. Posteriormente ingresó en emergencia por presentar dificultad respiratoria, tos seca, disminución de la conciencia, hipercalcemia, tomografía de tórax con patrón heterogéneo consolidativo en ambos pulmones y PCR en hisopado nasofaríngeo positivo a COVID-19. Recibió tratamiento de hidroxicloroquina, azitromicina, corticoides e ivermectina con pobre respuesta, rápido deterioro y fallece días después. La leucemia/linfoma T del adulto a edad temprana es rara y está relacionada con infecciones crónicas como strongyloides o tuberculosis, susceptible ante el padecimiento de COVID-19.

Adult T cell leukemia-lymphoma is a common malignancy with a poor prognosis in the elderly population. We present a 44-year-old woman from Ayacucho who was diagnosed with a lymphoma subtype of this disease and a human T-lymphotropic virus-I infection; she showed superior vena cava occlusive syndrome with systemic chemotherapy treatment under an adjusted-dose regimen with rituximab plus etoposide, prednisone, vincristine, cyclophosphamide and doxorubicin. Subsequently, she was admitted to the emergency service due to respiratory distress, dry cough, decreased consciousness, hipercalcemia, chest tomography with a heterogeneous consolidation pattern in both lungs and positive RT-PCR nasopharyngeal swab test for COVID-19. She received treatment with hydroxychloroquine, azithromycin, corticosteroids and ivermectin with a poor response, rapid deterioration and died later. Adult T cell leukemia-lymphoma at an early age is rare and is related to chronic infections such as strongyloides or tuberculosis, susceptible to COVID-19.

Linfoma no Hodgkin difuso de células B grande como linfoma primario de ovario. Presentación de caso / Diffuse large B-cell non-Hodgkin's lymphoma as primary ovarian lymphoma. Case presentation

Fundamento: Los linfomas primarios de ovario son poco frecuentes; el 1 % de estos se presenta en ovario y el 1.5 % de los tumores malignos de ovario son linfomas. Los tipos histológicos más frecuentes es el linfoma no Hodgkin difuso de células B grande y el BurKitt; el tratamiento consiste en cirugía combinada con quimioterapia. Objetivo: Reportar un caso de un linfoma no Hodgkin difuso de células B grande primario de ovario. Presentación de caso: Se presentó el caso de una paciente de 39 años de edad, con antecedentes patológicos personales de salud; la cual fue al cuerpo de guardia de ginecología por presentar dolor abdominal difuso que no se aliviaba con analgésicos. En la exploración física presentaba dolor a la palpación superficial y profunda en hipocondrio y fosa ilíaca derecha con masa tumoral palpable. Ecografía hacia proyección anexial derecha se observó una imagen de baja ecogenicidad y en la laparoscopia de urgencia se concluyó como una formación de aspecto tumoral que parecía corresponderse con ovario derecho. Se le realizó una histerectomía con doble anexectomía. El diagnóstico anatomopatológico fue un linfoma no Hodgkin primario de ovario. Conclusiones: La paciente del caso presentado tuvo una clínica oligosintomática y la confirmación de la enfermedad fue a partir de una muestra quirúrgica, lo que expresa que el diagnóstico del linfoma no Hodgkin de células B es difícil y aunque es poco frecuente siempre se debe tener en cuenta en el diagnóstico diferencial de las tumoraciones unilaterales de ovario.

Background: Primary ovarian lymphomas are uncommon, 1% of these malignancies occur in the ovary, and 1.5% of all ovarian malignancies are lymphomas. The most common histologic types are diffuse large B-cell non-Hodgkin's lymphoma and BurKitt's lymphoma; treatment consists of surgery combined with chemotherapy. Objective: To report a case of primary ovarian diffuse large B-cell non-Hodgkin lymphoma. Case presentation: A 39-year-old female case is presented, with a personal pathological history; she went to the gynecology emergency service because she presented diffuse abdominal pain that was not relieved by analgesics. Physical examination revealed superficial and deep pain on palpation in the hypochondrium and right illiac fossa with a palpable tumor mass. Right adnexal ultrasound showed an image of low echogenicity and at the emergency laparoscopy, it was diagnosed as a tumor-like formation that appeared to correspond to the right ovary. She underwent a hysterectomy with double adnexectomy. The anatomopathologic diagnosis was primary ovarian non-Hodgkin's lymphoma. Conclusions: The patient in the presented case had an oligosymptomatic clinical presentation. Confirmation of the disease was obtained from a surgical sample, which means that B-cell non-Hodgkin's lymphoma is difficult to diagnose and although it is uncommon, it should always be considered in the differential diagnosis of unilateral ovarian tumors.

Asociación Linfoma-Tuberculosis ganglionar: Un reto diagnóstico y terapéutico

La tuberculosis, una enfermedad infectocontagiosa, de amplia distribución mundial constituye en una de las causas de morbi-mortalidad más frecuentes en países subdesarrollados. Por otra parte, el linfoma de Hodgkin, una neoplasia linfoide maligna, poco frecuente, tiende a tener un diagnóstico diferencial amplio, incluyendo la tuberculosis extrapulmonar. Se presenta el caso de un paciente masculino de 10 años, procedente de un área endémica para tuberculosis, con cuadro de un mes de evolución de aumento de volumen abdominal, asociado a fiebre vespertina y pérdida de peso, tratado inicialmente como cuadro gastroentérico, sin embargo, con múltiples estudios se logró documentar Tuberculosis ganglionar y Linfoma de Hodgkin clásico en su variante esclerosis nodular en estadio IV b, constituyéndose en un reto diagnóstico y terapéutico.   (provisto por Infomedic International)

Tuberculosis, an infectious disease with a wide global distribution, constitutes one of the most frequent causes of morbidity and mortality in underdeveloped countries. On the other hand, Hodgkin lymphoma, a rare lymphoid malignancy, tends to have a wide differential diagnosis, including extrapulmonary tuberculosis. We present the case of a 10-year-old male patient, from an endemic area for tuberculosis, with a one-month history of increased abdominal volume, associated with evening fever and weight loss, initially treated as gastroenteric symptoms, however , with multiple studies it was possible to document lymph node tuberculosis and classical Hodgkin lymphoma in its variant nodular sclerosis in stage IV b, constituting a diagnostic and therapeutic challenge. (provided by Infomedic International)

Síndrome colestásico como forma de presentación de linfoma no Hodgkin. Reporte de un caso pediátrico / Cholestatic syndrome as a presentation of non-Hodgkin lymphoma. A pediatric case report

La ictericia colestásica se debe a la alteración de la secreción de bilirrubina conjugada; es una de las posibles causas la alteración del flujo biliar por obstrucción de la vía biliar extrahepática. El linfoma es la tercera neoplasia más frecuente en pediatría, mientras que los tumores pancreáticos son poco frecuentes y, en su mayoría, lesiones benignas. Las manifestaciones clínicas de los tumores de localización retroperitoneal son poco específicas y suelen ser tardías, por lo que la sospecha clínica debe ser alta. El objetivo del siguiente trabajo es presentar el caso de un niño de 7 años con síndrome colestásico en el que se halló un tumor en la cabeza del páncreas que comprimía la vía biliar extrahepática. El diagnóstico del tumor fue linfoma no Hodgkin (LNH). Se destaca la infrecuencia de este tumor en esta localización en la edad pediátrica

Cholestatic jaundice is due to an alteration in conjugated bilirubin secretion; a possible cause is an altered bile flow resulting from an obstruction of the extrahepatic bile duct. A lymphoma is the third most common neoplasm in pediatrics, while pancreatic tumors are rare and mostly benign. The clinical manifestations of retroperitoneal tumors are not very specific and are usually late, so a high level of clinical suspicion is required. The objective of this study is to describe the case of a 7-year-old boy with cholestatic syndrome with a tumor in the head of the pancreas compressing the extrahepatic bile duct. The tumor diagnosis was non-Hodgkin lymphoma (NHL). It is worth noting that the presence of a tumor in this location in pediatric age is uncommon