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Background: Cold agglutinin syndrome (CAS) is a hemolytic anemia mediated by antibodies, mainly IgM, whose maximum activity occurs at 4 °C. It happens secondary to infectious, autoimmune or neoplastic diseases, due to the formation of antibodies that cross-react against erythrocyte antigens, particularly of the I system. Here, we describe a case of CAS associated to Epstein-Barr virus (EBV) reactivation in a patient with primary human immunodeficiency virus (HIV) infection. Clinical case: 22-year old man with no medical history, hospitalized due to mononucleosis and anemic syndrome. Hemoglobin of 3.7 g/dL and elevation of lactate dehydrogenase were documented. In the peripheral blood smear it was observed spherocytosis, polychromasia and nucleated erythrocytes. EBV infection was confirmed with serology and viral load, as well as seronegative HIV infection with positive viral load. The C3d monospecific direct antiglobulin test was positive and an irregular antibody screening revealed the presence of an anti-I antibody. The patient received transfusion support and conservative treatment, with remission of the symptoms 2 weeks after admission. Conclusions: Cold agglutinin syndrome is a rare, potentially fatal complication of infectious mononucleosis, which should be considered in the face of findings suggestive of hemolysis in order to initiate support measures in a timely manner.

Introducción: el síndrome por aglutininas frías (SAF) es una anemia hemolítica mediada por anticuerpos principalmente de tipo IgM, cuya máxima actividad se da a 4 °C. Se presenta en el contexto de enfermedades infecciosas, autoinmunes o neoplásicas por la formación de anticuerpos que tienen reacción cruzada contra antígenos eritrocitarios, particularmente del sistema I. En este trabajo presentamos un caso de SAF asociado a reactivación del virus de Epstein-Barr (VEB) en un paciente con primoinfección por el virus de la inmunodeficiencia humana (VIH). Caso clínico: hombre de 22 años, sin antecedentes patológicos, hospitalizado por síndrome mononucleósico y anémico. Presentó hemoglobina de 3.7 g/dL y elevación de lactato deshidrogenasa. En el frotis de sangre periférica se observó esferocitosis, policromasia y eritrocitos nucleados. Se confirmó infección por VEB con serología y carga viral, así como infección por VIH seronegativa, con carga viral positiva. La prueba de antiglobulina directa monoespecífica a C3d fue positiva y el rastreo de anticuerpos irregulares demostró un anticuerpo anti-I. El paciente recibió soporte transfusional y tratamiento conservador, con remisión del cuadro a las 2 semanas de su ingreso. Conclusiones: el SAF es una complicación poco frecuente de la mononucleosis infecciosa, potencialmente mortal, la cual debe ser considerada ante hallazgos sugestivos de hemólisis con la finalidad de iniciar medidas de soporte de forma oportuna.

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Extranodal NK/T- cell lymphoma (ENKTCL) is an aggressive lymphoma driven by Epstein-Barr virus (EBV) infection in genetically susceptible individuals. It was historically called a lethal midline granuloma. Due to the angio-destructive nature of ENKTCL, lymphoma cells are often accompanied and masked by necrosis and dense inflammation in the biopsy. Further, the biopsy may show vasculitis, which can mimic granulomatosis with polyangiitis. Due to these masquerades, ENKTCL is often misdiagnosed in the biopsy. Several biopsies may be required to establish the diagnosis. We describe the clinical course and autopsy findings of a young female who presented with a hard-palate ulcer. Antemortem biopsies failed to establish the diagnosis. The autopsy revealed an advanced nasal subtype of Extranodal NK/T-cell lymphoma with dissemination to the kidneys, adrenals, liver, spleen, and small intestine.

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Introdução: A expressão da positividade do vírus Epstein-Barr nas células de Reed-Sternberg/Hodgkin e o impacto dessa relação na sobrevida do Linfoma de Hodgkin clássico (LHc) permanecem controversos. Objetivo: Avaliar a presença de Epstein Baar Vírus (EBV) em pacientes com LHc, associando com características anatomopatológicas e clínicas. Materiais e Métodos: Para determinar a presença de EBV, a partir de um material de biópsia de tecidual foi realizada analise por hibridização para RNA codificado para EBV (EBER) e imuno-histoquímica para proteína de membrana latente viral (LMP-1) de uma amostra de demanda de pacientes com diagnóstico entre janeiro de 2009 e janeiro de 2015. As características clínicas avaliadas foram as que compõem o Escore Prognóstico Internacional para estadiamento avançado e os fatores de risco desfavoráveis instituídos pelo Grupo Alemão de Estudos em Hodgkin para estadiamento limitado. Posteriormente, tais características foram relacionadas com a situação do vírus nas células tumorais. Resultados: Foram avaliadas as lâminas de biópsia de 29 pacientes com diagnóstico de Linfoma de Hodgkin. Em relação as características histológicas, o subtipo histológico de celularidade mista apresentou associação estatística com EBV positivo (p= 0,02). Além disso, a presença de EBV foi mais comum em pacientes com 45 anos ou mais (p= 0,07). Embora sem significância estatística, houve uma tendência de melhor sobrevida livre de eventos (p= 0,07), para os pacientes EBV positivo. Conclusão: O subtipo histológico de celularidade mista, apresentou uma relação estatisticamente significativa com a presença de EBV nas células tumorais, em conformidade com a literatura. São necessários estudo com maior número de casos para que se possa entender melhor a relação entre a presença de EBV e o prognóstico do LHc em nosso meio.

Introduction: The expression of Epstein-Barr virus positivity in Reed-Sternberg/Hodgkin cells and the impact of this relationship on survival in classical Hodgkin Lymphoma (cHL) remain controversial. Objective: To evaluate the presence of Epstein Baar Virus (EBV) in patients with cHL, associating it with anatomopathological and clinical characteristics.Materials and Methods: To determine the presence of EBV, analysis was carried out using a tissue biopsy material by hybridization for EBV-encoded RNA (EBER) and immunohistochemistry for viral latent membrane protein (LMP-1) from a demand sample of patients diagnosed between January 2009 and January 2015. The clinical characteristics evaluated were those that make up the International Prognostic Score for advanced stage and the unfavorable risk factors established by the German Hodgkin Study Group for limited stage. Subsequently, these characteristics were related to the status of the virus in tumor cells. Results: Biopsy slides from 29 patients diagnosed with Hodgkin's Lymphoma were evaluated. Regarding histological characteristics, the histological subtype of mixed cellularity showed a statistical association with positive EBV (p= 0.02). Furthermore, the presence of EBV was more common in patients aged 45 years or older (p= 0.07). Although not statistically significant, there was a trend towards better event-free survival (p= 0.07) for EBV positive patients. Conclusion: The histological subtype of mixed cellularity showed a statistically significant relationship with the presence of EBV in tumor cells, in accordance with the literature. Studies with a larger number of cases are needed to better understand the relationship between the presence of EBV and the prognosis of cHL in our country.

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ABSTRACT In equatorial Brazil, the association of Burkitt lymphoma and Epstein-Barr virus manifests at high rates. Here, we report, for the first time, amplifications of aurora kinase genes (AURKA/B) in a patient with a history of periodontal abscess and the presence of a remaining nodule, diagnosed with Burkitt lymphoma and Epstein-Barr virus, and /HIV positive. The patient was a 38-year-old man who presented with a 2-week-old severe jaw pain and a 3-day-old severe bilateral headache. He had a history of human papilloma virus. Interphase FISH analysis showed AURKA and AURKB amplification. The patient's condition worsened, progressing to death a month after the initial care. Changes in the MYCC and AURKA pathways are directly associated with genomic instability. Thus, MYCC rearrangements and higher expression of AURKA/B may be associated with therapy resistance, highlighting the importance of AURKA/B evaluation in Burkitt lymphoma.

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ABSTRACT Extranodal NK/T- cell lymphoma (ENKTCL) is an aggressive lymphoma driven by Epstein-Barr virus (EBV) infection in genetically susceptible individuals. It was historically called a lethal midline granuloma. Due to the angio-destructive nature of ENKTCL, lymphoma cells are often accompanied and masked by necrosis and dense inflammation in the biopsy. Further, the biopsy may show vasculitis, which can mimic granulomatosis with polyangiitis. Due to these masquerades, ENKTCL is often misdiagnosed in the biopsy. Several biopsies may be required to establish the diagnosis. We describe the clinical course and autopsy findings of a young female who presented with a hard-palate ulcer. Antemortem biopsies failed to establish the diagnosis. The autopsy revealed an advanced nasal subtype of Extranodal NK/T-cell lymphoma with dissemination to the kidneys, adrenals, liver, spleen, and small intestine.

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Resumen La mononucleosis infecciosa es un síndrome clínico de causa viral que generalmente tiene un curso benigno y autolimitado, caracterizado por fiebre, faringoamigdalitis y adenomegalias generalizadas, siendo la afectación de órganos o sistemas algo inusual. Se presenta el caso de una niña de ocho años con un síndrome mononucleósico por virus de Epstein-Barr y Citomegalovirus, con un síndrome nefrítico agudo hipocomplementémico secundario, sin deterioro de la función renal y evolución clínica favorable. La ausencia de un período de latencia entre el proceso infeccioso y la aparición de los síntomas de síndrome nefrítico, es un elemento clave en el diagnóstico diferencial con el síndrome nefrítico agudo postestreptocócico, que es la causa más común en niños.

Abstract Infectious mononucleosis is a clinical syndrome of viral cause that generally has a benign clinical picture, characterized by fever, pharyngotonsillitis, and generalized lymphadenopathy, being the affectation of organ or systems something unusual. We present the case of an eight-year-old girl with a mononucleosis syndrome due to Epstein-Barr virus and Cytomegalovirus, with a secondary hypocomplementemia acute nephritic syndrome, without deterioration of renal function and favorable clinical evolution. The absence of a latency period between the infectious process and the appearance of nephritic syndrome symptoms are a key element in the differential diagnosis with post-streptococcal acute nephritic syndrome, which is the most common cause in children.

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Introduction: LMP-1 (latent membrane protein 1) is a membrane protein found in EBV and can be identified through immunohistochemistry. Objectives: To evaluate the prevalence of EBV virus in tonsillar tissue samples correlating with tonsil size, age and gender. Methods: An LMP-1 immunohistochemical study was performed in slides from patients with tonsil hyperplasia. Results: The sample consisted of 120 slides, 66 were from male. The average tonsillar size was 6.0 cm2 (1.5-14.0) and the average age was 6.5 years (2-18). Overall, 72 patients were positive for the virus, the majority being male (51%) and preschoolers (51.4%). For comparative analysis, the sample was divided: group 1 (positive immunohistochemistry) and group 2 (negative immunohistochemistry). The average age was 6.74 years (± 4.14) and 6.19 years (± 3.82) for group 1 and 2, respectively. Group 1 had 37 preschoolers, 23 school-age children and 12 adolescents. Group 2 had 28 preschoolers, 14 school-age children and 6 adolescents (p=0.71). To evaluate the presence of virus, the slides were divided according to area: G (≥6cm²) and P (<6cm²). In G group, 54 slides were positive, while in P group, 18 were positive. Group 1 presented 54 of G group slides and 18 of P group slides, with no statistical difference, but with a tendency to positivity (p=0.09). Conclusion: Most of the sample was positive for the presence of Epstein-Barr virus. There was no difference in the virus presence or absence when correlated with the tonsillar size, age or sex

Introdução: LMP-1 (proteína latente de membrana-1) é uma proteína de membrana encontrada no Epstein-Barr vírus (EBV) e que pode ser identificadaatravés da imunoistoquímica. Objetivo: Avaliar a prevalência do EBV em amostras de tecido tonsilar correlacionando com o tamanho da tonsila, idade e sexo. Método: Realizou-se um estudo imunoistoquímico de LMP-1 para EBV em lâminas de pacientes com hiperplasia de tonsilas. Resultados: A amostra foi composta por 120 lâminas, sendo 66 (55%) de pacientes do sexo masculino. O tamanho médio tonsilar foi 6,0 cm2 (1,5-14,0) e a idade média 6,5 anos (2-18). Ao todo, 72 pacientes (60%) tiveram positividade para o vírus, a maioria meninos (51%) e pré-escolares (51,4%). A amostra foi dividida em: grupo 1 (imunoistoquímica positiva) e grupo 2 (imunoistoquímica negativa). A idade média foi de 6,74 anos (±4,14) e 6,19 anos (±3,82), para o grupo 1 e 2, respectivamente. O grupo 1 apresentou 37 pré-escolares, 23 escolares e 12 adolescentes. O grupo 2 foi composto por 28 pré-escolares, 14 escolares e 6 adolescentes, sem diferença estatística (p=0,71). Para avaliar a presença do EBV, as lâminas foram divididas de acordo com a área: G (≥6 cm²) e P (<6 cm²). No grupo G, 54 lâminas foram positivas, enquanto no grupo P, 18, sem diferença estatística, mas com tendência a positividade (p=0,09). Conclusão: A maior parte da amostra foi positiva para o Epstein-Barr vírus. Não houve diferença significante na positividade ou não do vírus quando correlacionada com o tamanho tonsilar, a idade e o sexo dos pacientes.

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Idiopathic CD4 lymphocytopenia (ICL) not related to HIV is an infrequent and severe condition with no etiology defined until now. The concomitant presence of an underlying disease, especially an oncohematological process, could be related to the immune physiopathology and the development of the immunosuppressive state. On the other hand, Epstein Barr virus is a well-known oncogenic pathogen described in the development of several types of lymphoma which might be reactivated in the ICL. There is still no specific treatment for this syndrome, so the therapeutic scope for these patients is the treatment of opportunistic diseases and the administration of specific antimicrobials as prophylaxis. We present a patient with an uncommon association of an ICL and an extranodal T/NK lymphoma with detection of VEB nuclear RNA by in situ hybridization (EBER). Diagnosis was challenging which led the health team to carry out many studies over several months.

La linfocitopenia CD4 idiopática (ICL) no relacionada al HIV es una condición grave e infrecuente sin una etiología aún definida. La presencia de una enfermedad subyacente, especialmente un proceso oncohematológico, podría tener relación en la fisiopatología del proceso inmunológico. Por otro lado, el virus Epstein Barr (VEB) es bien conocido por ser un patógeno oncogénico descrito en el desarrollo de diversos tipos de linfomas, el cual podría ser reactivado en estados de inmunosupresión severa. No existe aún un tratamiento específico para este síndrome, por lo que el objetivo terapéutico en estos pacientes radica en el manejo profiláctico y activo de las distintas enfermedades oportunistas ante las cuales son susceptibles. Se presenta un paciente con un déficit grave de linfocitos CD4 de causa idiopática, y un diagnóstico posterior de linfoma T/NK extraganglionar con detección de RNA nuclear de VEB por hibridización in situ (EBER), una asociación poco descrita en la literatura médica.

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Abstract Idiopathic CD4 lymphocytopenia (ICL) not related to HIV is an infrequent and severe condition with no etiology defined until now. The concomitant presence of an underlying disease, especially an oncohematological process, could be related to the immune physiopathology and the development of the im munosuppressive state. On the other hand, Epstein Barr virus is a well-known oncogenic pathogen described in the development of several types of lymphoma which might be reactivated in the ICL. There is still no specific treatment for this syndrome, so the therapeutic scope for these patients is the treatment of opportunistic diseases and the administration of specific antimicrobials as prophylaxis. We present a patient with an uncommon asso ciation of an ICL and an extranodal T/NK lymphoma with detection of VEB nuclear RNA by in situ hybridization (EBER). Diagnosis was challenging which led the health team to carry out many studies over several months

Resumen La linfocitopenia CD4 idiopática (ICL) no relacionada al HIV es una condición grave e infrecuente sin una etiología aún definida. La presencia de una enfermedad subyacente, especialmente un proceso oncohematológico, podría tener relación en la fisiopatología del proceso inmunológico. Por otro lado, el virus Epstein Barr (VEB) es bien conocido por ser un patógeno oncogénico descrito en el desarrollo de diversos tipos de linfomas, el cual podría ser reactivado en estados de inmunosupresión severa. No existe aún un tratamiento específico para este síndro me, por lo que el objetivo terapéutico en estos pacientes radica en el manejo profiláctico y activo de las distintas enfermedades oportunistas ante las cuales son susceptibles. Se presenta un paciente con un déficit grave de linfocitos CD4 de causa idiopática, y un diagnóstico posterior de linfoma T/NK extraganglionar con detección de RNA nuclear de VEB por hibridización in situ (EBER), una asociación poco descrita en la literatura médica.

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Extranodal NK/T-cell lymphoma, nasal type (ENKTL-NT) is a rare type of Non-Hodgkin's lymphoma, which usually presents with extranodal involvement and affects the nasal/upper aerodigestive tract in the classical presentation. Herein, we report the case of a 31-year-old, previously healthy, male patient diagnosed with ENKTL-NT with the involvement of the lung parenchyma and heart. Unfortunately, due to the rapid disease progression, the diagnosis was performed only at the autopsy. The authors highlight the rare clinical presentation of this type of lymphoma, as well as the challenging anatomopathological diagnosis in necrotic samples.

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Abstract Epstein Barr virus-associated smooth muscle tumors are an uncommon neoplasm that occurs in immunosuppressed patients of any age. Usually, it presents as multifocal tumors mainly in the spinal cord, epidural region, gastrointestinal tract and liver, upper respiratory tract and skin, the latest with few cases reported in the literature and related with human immunodeficiency virus infection and acquired immune deficiency syndrome. The authors present the first case of a Colombian adult patient with human immunodeficiency virus infection and multifocal Epstein Barr virus-associated smooth muscle tumors in the skin and epidural region, confirmed by histopathology, immunohistochemistry and in situ hybridization studies.

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Epstein Barr virus-associated smooth muscle tumors are an uncommon neoplasm that occurs in immunosuppressed patients of any age. Usually, it presents as multifocal tumors mainly in the spinal cord, epidural region, gastrointestinal tract and liver, upper respiratory tract and skin, the latest with few cases reported in the literature and related with human immunodeficiency virus infection and acquired immune deficiency syndrome. The authors present the first case of a Colombian adult patient with human immunodeficiency virus infection and multifocal Epstein Barr virus-associated smooth muscle tumors in the skin and epidural region, confirmed by histopathology, immunohistochemistry and in situ hybridization studies.

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A ocorrência de úlceras genitais em adolescentes e mulheres jovens tem impacto emocional para as pacientes e seus familiares, pela frequente associação com uma possível etiologia de transmissão sexual. Porém, úlcera de Lipschütz e síndrome de Behçet não têm etiologia infecciosa e devem ser lembradas como possíveis diagnósticos diferenciais. O diagnóstico dessas duas patologias é clínico e pode ser desafiador. Dessa forma, foi realizada uma revisão na literatura com o objetivo de comparar as duas entidades. A úlcera de Lipschütz é causada por uma vasculite local e caracteriza-se pelo surgimento súbito de úlceras na vulva ou vagina inferior. Já a doença de Behçet é causada por vasculite sistêmica, com episódios de remissão e exacerbação, que pode envolver quase todos os sistemas orgânicos. Em ambos os casos, é essencial o referenciamento para reumatologia. O tratamento objetiva suprimir exacerbações, controlar a dor e prevenir infecção secundária.(AU)

The occurrence of genital ulcers in adolescents and young women have an emotional impact for the patient and their families, due to the frequent association of its etiology with a sexually transmitted disease. However, Lipschütz ulcer and Behçet's syndrome do not have an infectious etiology and should be remembered as a possible differential diagnoses. As the diagnosis of these two pathologies is clinical and can be challenging, a review of literature was carried out. The objective of this review of literature was to compare both diseases. Lipschütz ulcer is caused by local vasculitis and is characterized by the sudden appearance of ulcers in the vulva or lower vagina. Behçet's syndrome is caused by systemic vasculitis, with episodes of remission and exacerbation, which can affect almost all organ systems. In both cases, referral to rheumatology is essential. Treatment aims to suppress exacerbations, control pain and prevent secondary infection.(AU)

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Extranodal NK/T-cell lymphoma, nasal type (ENKTL-NT) is a rare type of Non-Hodgkin's lymphoma, which usually presents with extranodal involvement and affects the nasal/upper aerodigestive tract in the classical presentation. Herein, we report the case of a 31-year-old, previously healthy, male patient diagnosed with ENKTL-NT with the involvement of the lung parenchyma and heart. Unfortunately, due to the rapid disease progression, the diagnosis was performed only at the autopsy. The authors highlight the rare clinical presentation of this type of lymphoma, as well as the challenging anatomopathological diagnosis in necrotic samples.

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Introducción:El síndrome hemofagocítico (SHF) es reconocido como un conjunto de signos clínicos y hallazgos laboratoriales que tienen un grave compromiso en la salud y vitalidad de los niños con una incidencia de 1.2 casos/millón/año. Puede pasar subdiagnosticado y confundido con sepsis de foco inespecífico Caso clínico:Niño de 4 años de edad, sin antecedentes de importancia. Ingresado desde el servicio de emergencia por presentar 20 días de fiebre y dolor abdominal. Requirió intubación por franca falla respiratoria y el ingreso a la Unidad de Cuidados Intensivos Pediátricos. Con hipotensión e insuficiencia hepática, pancitopeniay esplenomegalia. Evolución: Se descartaron infecciones bacterianas con policultivos, SARS-Cov 2negativo,se descartaron inmunodeficiencias congénitas y adquiridas.TORCHnegativo, VDRL no reactivo.La prueba de Epstein Barr fue positivo para IgM.Se determinó endocarditis con derrame pericárdico global. Estudio de biopsia medular normocromía, normocitosis, pancitopenia y blastos <5%, sin infiltración tumoral. Se estableció el Diagnóstico de SHFse inicióciclosporina y corticoterapia.Requirió ventilación mecánica por 20 días con período de pronación de 36 horas. Fue dado de alta a pediatríay posteriormente a domicilio, para control por consulta externa. Conclusión: El diagnóstico del SHF es inusual y subestimado al momento de la evaluación clínica. En el presente reporte se asocia a la presencia del Virus Epstein Barr

Introduction: Hemophagocytic syndrome (HPS) is recognized as a set of clinical signs and laboratory findings that have a serious compromise on the health and vitality of children with an incidence of 1.2 cases / million / year. It can be underdiagnosed and confused with sepsis with a non-specific focus. Clinical case: 4-year-old boy, with no significant history. Admitted from the emergency service due to 20 days of fever and abdominal pain. She required intubation due to frank respiratory failureand admission to the Pediatric Intensive Care Unit. With hypotension and liver failure, pancytopenia and splenomegaly. Evolution: Bacterial infections were ruled out with polycultures, SARS-Cov 2 negative, congenital and acquired immunodeficiencies were ruled out. Negative TORCH, non-reactive VDRL. The Epstein Barr test was positive for IgM. Endocarditis with global pericardial effusion was determined. Medullary biopsy study normochromia, normocytosis, pancytopenia, and blasts <5%, without tumor infiltration. The diagnosis of SHF was established, cyclosporine and corticosteroid therapy were started. He required mechanical ventilation for 20 days with a 36-hour pronation period. He was discharged to pediatrics and later at home, for outpatient control. Conclusion: The diagnosis of HHS is unusual and underestimated at the time of clinical evaluation. In this report it is associated with the presence of the Epstein Barr Virus

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INTRODUCTION: The role of viral infections in the pathogenesis of autoimmune diseases has long been suggested, but little evidence is available. OBJECTIVE: This study aimed to evaluate an association between EBV and CMV and the presence of rheumatoid arthritis and its association with Sjögren's Syndrome. PATIENTS AND METHOD: A case-control study was performed with 227 patients divided in RA (n = 99), RA/SS (n = 20), and C (n = 128). Resting salivary flow rate and Schirmer's test were performed; minor salivary gland biopsy was indicated in the case of suspected Sjögren's syndrome. CMV and EBV viral loads were quantified in peripheral blood, and their presence in glandular tissue samples was evaluated by in situ hybridization (EBV) and immunohistochemistry (CMV). RESULTS: EBV was more frequent in RA and RA/SS than in C (P < .000007). No correlation with clinical markers (P > .05) or between RA and RA/SS was found (P > .05). A higher number of EBV/DNA copies were found in RA (158.52 copies/µL) and RA/SS (99.24 copies/µL) (P = .739). EBV/DNA was associated with the Schirmer test (P = .0231). CMV was detected in one patient of the RA group. None of the viruses were detected in biopsies of minor salivary glands. CONCLUSIONS: Detection of EBV/DNA in peripheral blood was associated with RA regardless of the presence of SS.

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Introduction: The Epstein-Barr virus (EBV) is an ubiquitous and oncogenic virus associated with the development of diseases such as infectious mononucleosis, Burkitt's lymphoma, nasopharyngeal carcinoma, and other neoplasms. Currently, two types are recognized: EBV-1 and EBV-2, which have genetic differences with their EBNA nuclear antigens. Likewise, due to the high degree of heterogeneity and variability found in the LMP1 protein of the virus, variants associated with pathogenesis or specific geographic regions have been described. Objective: To identify and characterize molecularly EBV variants detected in the oral cavity of 84 adolescents in Cali, Colombia. Materials and methods: Conventional PCR amplification, purification, and sequencing of the gen EBNA3C were carried out to typify the virus and the C-ter domain of the LMP1 protein to identify variants. We also conducted a phylogenetic and nucleotide variant analysis of the obtained sequences versus pathogenic or geographic variants reported in GenBank-NCBI. Results: The predominant viral subtype was EBV-1 (79%); 72.6% was grouped with the pathogenic variant Raji, derived from B lymphocytes of a patient with Burkitt>s lymphoma, 13.7% was related to a variant of Mediterranean origin, and 13.7% was not grouped with any of the reference variants. Conclusions: This is the first time that variants of LMP1-EBV have been identified in Cali, Colombia. Additional studies are necessary to characterize the unidentified variant and to determine if it is pathogenic or if it is just an isolate present in the city of Cali.

Introducción. El virus de Epstein-Barr (EBV) es un virus ubicuo y oncogénico, asociado con el desarrollo de enfermedades como la mononucleosis infecciosa, el linfoma de Burkitt, el carcinoma nasofaríngeo y otras neoplasias. Actualmente, se reconocen dos subtipos: EBV-1 y EBV- 2, que tienen diferencias genéticas con sus antígenos nucleares (Epstein-Barr Nuclear Antigens, EBNA). Debido a la gran heterogeneidad y variabilidad encontradas en la proteína LMP1 del virus, se han descrito variantes asociadas con ciertas enfermedades o con regiones geográficas específicas. Objetivo. Identificar y caracterizar molecularmente las variantes del EBV detectadas en la cavidad oral de 84 adolescentes de Cali, Colombia. Materiales y métodos. Se hizo la amplificación por reacción en cadena de la polimerasa (Polymerase Chain Reaction, PCR) convencional, así como la purificación y la secuenciación del gen EBNA3C se realizó para subtipificar el virus y del dominio C-ter de la proteína LMP1 para identificar variantes. Además, se llevó a cabo un análisis filogenético y de variantes nucleotídicas de las secuencias obtenidas comparadas con variantes patogénicas y geográficas reportadas en el GenBank (National Center for Biotechnology Information, NCBI). Resultados. El subtipo viral predominante fue el EBV-1 (79 %); el 72,6 % se agrupó con la variante patogénica Raji, derivada de linfocitos B de un paciente con linfoma de Burkitt; el 13,7 % se relacionó con una variante de origen geográfico del Mediterráneo y otro 13,7 % no se agrupó con ninguna de las variantes de referencia. Conclusiones. Este es el primer estudio que reporta variantes del gen LMP1-EBV en Cali, Colombia. Se requieren nuevos estudios para caracterizar la variante sin identificar y determinar si es patogénica o si es una variante geográfica presente exclusivamente en la ciudad.

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Introducción. El virus de Epstein-Barr (EBV) es un virus ubicuo y oncogénico, asociado con el desarrollo de enfermedades como la mononucleosis infecciosa, el linfoma de Burkitt, el carcinoma nasofaríngeo y otras neoplasias. Actualmente, se reconocen dos subtipos: EBV-1 y EBV- 2, que tienen diferencias genéticas con sus antígenos nucleares (Epstein-Barr Nuclear Antigens, EBNA). Debido a la gran heterogeneidad y variabilidad encontradas en la proteína LMP1 del virus, se han descrito variantes asociadas con ciertas enfermedades o con regiones geográficas específicas. Objetivo. Identificar y caracterizar molecularmente las variantes del EBV detectadas en la cavidad oral de 84 adolescentes de Cali, Colombia. Materiales y métodos. Se hizo la amplificación por reacción en cadena de la polimerasa (Polymerase Chain Reaction, PCR) convencional, así como la purificación y la secuenciación del gen EBNA3C se realizó para subtipificar el virus y del dominio C-ter de la proteína LMP1 para identificar variantes. Además, se llevó a cabo un análisis filogenético y de variantes nucleotídicas de las secuencias obtenidas comparadas con variantes patogénicas y geográficas reportadas en el GenBank (National Center for Biotechnology Information, NCBI). Resultados. El subtipo viral predominante fue el EBV-1 (79 %); el 72,6 % se agrupó con la variante patogénica Raji, derivada de linfocitos B de un paciente con linfoma de Burkitt; el 13,7 % se relacionó con una variante de origen geográfico del Mediterráneo y otro 13,7 % no se agrupó con ninguna de las variantes de referencia. Conclusiones. Este es el primer estudio que reporta variantes del gen LMP1-EBV en Cali, Colombia. Se requieren nuevos estudios para caracterizar la variante sin identificar y determinar si es patogénica o si es una variante geográfica presente exclusivamente en la ciudad.

Introduction: The Epstein-Barr virus (EBV) is an ubiquitous and oncogenic virus associated with the development of diseases such as infectious mononucleosis, Burkitt's lymphoma, nasopharyngeal carcinoma, and other neoplasms. Currently, two types are recognized: EBV-1 and EBV-2, which have genetic differences with their EBNA nuclear antigens. Likewise, due to the high degree of heterogeneity and variability found in the LMP1 protein of the virus, variants associated with pathogenesis or specific geographic regions have been described. Objective: To identify and characterize molecularly EBV variants detected in the oral cavity of 84 adolescents in Cali, Colombia. Materials and methods: Conventional PCR amplification, purification, and sequencing of the gen EBNA3C were carried out to typify the virus and the C-ter domain of the LMP1 protein to identify variants. We also conducted a phylogenetic and nucleotide variant analysis of the obtained sequences versus pathogenic or geographic variants reported in GenBank-NCBI. Results: The predominant viral subtype was EBV-1 (79%); 72.6% was grouped with the pathogenic variant Raji, derived from B lymphocytes of a patient with Burkitt>s lymphoma, 13.7% was related to a variant of Mediterranean origin, and 13.7% was not grouped with any of the reference variants. Conclusions: This is the first time that variants of LMP1-EBV have been identified in Cali, Colombia. Additional studies are necessary to characterize the unidentified variant and to determine if it is pathogenic or if it is just an isolate present in the city of Cali.

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RESUMEN El linfoma extranodal de células T/NK extranasal (NKTL) primario del tracto gastrointestinal es poco frecuente y tiene carácter agresivo. Presentamos el caso de un paciente inmunocompetente de 51 años que ingresa por dolor abdominal de dos meses de evolución. En la colonoscopía, se hallaron ulceraciones ileales, por lo que se le realizaron estudios para descartar enfermedad de Crohn y tuberculosis intestinal, posteriormente presentó obstrucción intestinal. En la laparotomía exploratoria, se encontró un conglomerado ganglionar en mesenterio. La anatomía patológica confirmó el diagnóstico de NKTL primario en íleon. Se inició quimioterapia y el paciente pidió alta voluntaria dada su precaria condición. Dos meses después del alta el paciente fallece. Este linfoma ha sido reportado principalmente en Asia y posiblemente sea el primer caso reportado en Perú. Tiene un pronóstico funesto con una supervivencia global de ocho meses. Por ello, es necesario un diagnóstico precoz e iniciar la terapia oportunamente.

ABSTRACT Primary extranodal Natural Killer / T cell lymphoma (NKTL) on gastrointestinal tract is an uncommon and aggressive neoplasm. We present the case of a 51-year-old immunocompetent patient with a 2-month history of abdominal pain. Colonoscopy findings showed ileal ulcerations, so studies were carried out to rule out Crohn's disease and intestinal tuberculosis. Later, he developed intestinal obstruction. Exploratory laparotomy found a nodal conglomerate in the mesentery. Anatomical pathology confirmed the diagnosis of primary NKTL on the ileum. Chemotherapy was initiated but the patient asked for voluntary discharge because of his precarious condition. Two months after discharge the patient died. This lymphoma has been reported mainly in Asia and is possibly the first case in Peru. It has a dismal prognosis with overall survival of 8 months. Therefore, it is necessary to get an early diagnosis and begin therapy in a timely manner.

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RESUMEN Introducción. La infección por virus de Epstein-Barr (VEB) suele ser asintomática y persiste durante toda la vida. La afectación ocular es infrecuente, y aunque existen informes de casos, ninguno de ellos proviene de Colombia o Latinoamérica. Presentación del caso. Paciente masculino inmunocompetente con vasculitis retiniana unilateral generalizada, con vasos sin sangre temporales e inferonasales en la periferia, hemorragias intrarretinianas, vitritis intensa y desprendimiento de retina. La presencia de VEB se definió con una técnica de reacción en cadena de la poli-merasa en humor vítreo. El paciente recuperó la agudeza visual con el tratamiento oral antiviral convencional. Discusión. La afectación ocular asociada con el VEB se describe en pacientes inmunocomprometidos, en especial con infección por virus de inmunodeficiencia humana donde puede haber afectación retiniana. En este caso particular se discute la presencia de esta patología en pacientes inmunocompetentes. Conclusiones. La vasculitis retiniana es una entidad rara con mayor asociación a la inmuno-depresión. No existe un algoritmo de diagnóstico de esta enfermedad y la afectación ocular puede ser variable; tampoco existen líneas estándar de tratamiento. La evidencia reportada en el presente caso podría justificar estudios en pacientes seleccionados que muestran un compromiso de la agudeza visual sin una etiología establecida.

ABSTRACT Introduction: Epstein - Barr virus (EBV) infection is usually asymptomatic and persists throughout life. Eye involvement is rare, and even though there are some case reports, none of them comes from Colombia or Latin America. Case presentation: Immunocompetent young man with generalized unilateral retinal vasculitis, temporal and inferonasal bloodless vessels in the periphery, intraretinal hemorrhages, intense vitritis and retinal detachment. Epstein-Barr virus presence was determined using a polymerase chain reaction technique in vitreous humor. The patient recovered visual acuity with conventional antiviral oral treatment. Discussion: Eye involvement associated with Epstein-Barr virus is observed in immunocompromised patients, especially with HIV infection, where retinal involvement may occur. This case reports the presence of this pathology in an immunocompetent patient. Conclusions: Retinal vasculitis is a rare entity, frequently associated with immunocompromise. There is no diagnostic algorithm for this disease and eye involvement may be variable; there are no standard lines of treatment either. The evidence reported here explains the need for studies in selected patients showing visual acuity involvement without an established etiology.