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.

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 gástrico primario a células T, no asociado a HTLV1, con presentación inicial de fractura patológica. Reporte de caso

El linfoma de células T en adultos (ATLL) es una neoplasia agresiva de linfocitos T, por lo general asociada con el virus linfotrópico T humano tipo 1 (HTLV-1), de presentación clínica abigarrada. Los linfomas gástricos primarios son generalmente linfoma no Hodgkin (NH) tipo B, y un mínimo porcentaje por linfocitos T. Es escasa la literatura sobre linfomas gástricos primarios por células T con HTLV-1 Negativo y que hacen metástasis ósea. Para ilustrar esta situación, se presenta el caso de un adulto de 41 años, que ingresa por una fractura patológica. A los 15 días presenta diarrea, distensión abdominal, y una endoscopia alta encuentra "Lesiones ulceradas gástricas, aspecto neoproliferativo". La biopsia informó Linfoma No Hodgkin a células maduras linfocito T; y la prueba de HTLV1 fue negativa. Se realizaron tres sesiones de quimioterapia con esquema CHOEP. Hubo respuesta favorable, saliendo de alta; sin embargo, no retorna para proseguir la terapia. El paciente regresó dos meses después en mal estado general; luego presentó falla multiorgánica, produciéndose su deceso.

Adult T-cell lymphoma (ATLL) is an aggressive T-cell neoplasm, usually associated with human T-lymphotropic virus type 1 (HTLV-1), with a variegated clinical presentation. Primary gastric lymphomas are generally non-Hodgkin lymphoma (NH) type B, and a minimal percentage are due to T lymphocytes. There is little literature on primary gastric lymphomas due to HTLV-1 Negative T cells that metastasize to bone. To illustrate this situation, the case of a 41-year-old adult who is admitted for a pathological fracture is presented. 15 days later, he developed diarrhea and abdominal distension, and an upper endoscopy found "gastric ulcerated lesions, neoproliferative appearance". The Biopsy reported Non-Hodgkin Lymphoma to mature T lymphocyte cells; and the HTLV1 test was negative. Three chemotherapy sessions were performed with the CHOEP scheme. There was a favorable response, and he was discharged; However, he did not return to continue therapy. The patient returned 2 months later in poor general condition. He then presented multiple organ failure, resulting in his death.

Distribución geográfica y tipo de infección del virus linfotrópico T humano en pacientes peruanos 2019-2021

En el presente estudio describimos y caracterizamos la distribución geográfica de los casos positivos confirmados a HTLV-1 y 2 de pacientes peruanos con diagnóstico presuntivo entre 2019 y 2021. De un total de 555 muestras positivas confirmadas, 546 (98,4%) fueron HTLV-1 y 9 (1,6%) HTLV-2. Además, 22 de 24 departamentos del Perú presentaron casos de HTLV-1, siendo los principales motivos de solicitud de confirmación diagnóstica: aspirante a donar sangre con prueba de tamizaje reactivo, sospecha de leucemia/linfoma y paraparesia espástica tropical. Los resultados reflejan que la identificación de los puntos críticos constituye una brecha persistente respecto al diagnóstico, siendo cruciales para reducir el número de nuevos casos en Perú.

In the present study we describe and characterize the geographic distribution of HTLV-1 and 2 positive cases from Peruvian patients with presumptive diagnosis 2019 - 2021. Of a total of 555 confirmed positive samples, 546 (98.4%) were HTLV-1 and 9 (1.6%) HTLV-2. In addition, 22 of 24 departments of Peru presented cases of HTLV-1. The main reasons for requesting a confirmatory diagnosis being: aspiring to donate blood with a reactive screening test, suspicion of leukemia/ lymphoma and tropical spastic paraparesis. The results reflect that the identification of critical points constitutes a persistent gap regarding the diagnosis, being crucial to reduce the number of new cases in Peru.

Basic zipper protein (HBZ): biomarcador diagnóstico y de seguimiento de leucemia/linfoma T del adulto / Basic zipper protein ( HBZ ) : diagnostic and follow - up biomarker of adult T - cell leukemia/lymphoma

Introducción: El virus linfotrópico de células T humanas tipo 1 es el retrovirus causal de la leucemia-linfoma de células T. Su escaso diagnóstico en fases agudas hace que encontrar un biomarcador acertado para conocer el desarrollo de la leucemia sea de interés clínico y científico. Objetivo: Presentar la proteína basic zipper protein como marcador diagnóstico y de seguimiento al tratamiento de la leucemia-linfoma de células T. Métodos: Se consultó material académico en diferentes bases de datos científicas tales como PubMed, Springerlink, Proquest y Sciencedirect. Se tuvieron en cuenta criterios como datos, efectividad en la identificación y amplificación de basic zipper protein, reconocimiento de las fases del virus linfotrópico de células T, epidemiología y mecanismos moleculares en la patogenia del virus. Análisis y síntesis de la información: La basic zipper protein es una proteína del virus linfotrópico de células T indispensable para el proceso de leucemogénesis, capaz de interactuar con factores endógenos del huésped, lo que la convierte en marcador de la enfermedad. Conclusiones: La basic zipper protein cuenta con características que la perfilan para ser el biomarcador con mayor predicción de la enfermedad por su estabilidad e importancia en la leucemogénesis. Además, el nivel de ARNm de la basic zipper protein es mayor en leucemia-linfoma de células T.

Introduction: Human T-cell lymphotropic virus type 1 is the retrovirus that causes T-cell leukemia-lymphoma. Its scarce diagnosis in acute phases means that finding an accurate biomarker to determine the development of leukemia is of clinical and scientific interest. Objective: To present the basic zipper protein as a diagnostic and follow-up marker for treatment of T-cell leukemia-lymphoma. Methods: Academic material was consulted in different scientific databases such as PubMed, Springerlink, Proquest and Sciencedirect. Criteria such as: data, effectiveness in the identification and amplification of basic zipper protein, recognition of the phases of the T-cell lymphotropic virus, epidemiology and molecular mechanisms in the pathogenesis of the virus were taken into account. Analysis and synthesis of the information: The basic zipper protein is a protein of the T-cell lymphotropic virus essential for the leukemogenesis process, capable of interacting with endogenous factors of the host, which makes it a marker of the disease. Conclusions: The Basic zipper protein has characteristics that outline it to be the biomarker with the highest prediction of the disease due to its stability and importance in leukemogenesis. In addition, the mRNA level of the basic zipper protein is higher in T-cell leukemia-lymphoma.

Pronostico desfavorable de linfoma t/nk: reporte de casos

El linfoma de células T/NK tiene una frecuencia de presentación inusual, caracterizado por una progresión rápida y de mal pronóstico. Ocurrencia aludida a regiones de Asia y Latinoamérica por la fuerte incidencia de infección por el virus de Epstein- Barr. Su presentación puede ser nasal o en otra localización. Los esquemas de tratamiento suelen conllevar respuestas insuficientes, empero protocolos con base en L-Asparaginasa reflejarían mejores resultados. El presente reporte corresponde a 4 casos de este tipo de linfoma, 2 de tipo nasal y 2 de diversa presentación. Todos evolucionaron desfavorablemente, en parte por el mal pronóstico atribuido y también por el diagnóstico suscitado en etapas avanzadas de enfermedad concatenadas a dificultades económicas para sostener el tratamiento, factores intervinientes en nuestro medio.

NK/T cell lymphoma is an unusual neoplasm, characterized by fast progression and poor prognosis. It is present in regions of Asia and Latin America associated to a high incidence of Epstein-Barr virus infection. Its presentation can be nasal or in another location. Treatment schemes usually lead to insufficient responses, however protocols based on L-Asparaginase would reflect better results. This report concerns 4 cases of this lymphoma, 2 nasal type and 2 of a different presentation. Patients responses were unfavorable, partly due to the poor prognosis att r ibuted and also due to the diagnosis raised in advanced stages of the disease as well as to economic difficulties to sustain treatment, intervening factors in our country.

Linfoma angioinmunoblástico de células T como imitador de lupus eritematoso sistémico

El linfoma angioinmunoblástico de células T (LAIT) es un linfoma no Hodgkin poco frecuente, puede imitar a enfermedades autoinmunes y es de pobre pronóstico. Se presenta el caso de una mujer de 36 años con 3 años de enfermedad caracterizada por fiebre, artralgias y baja de peso. La paciente fue diagnosticada inicialmente como lupus eritematoso sistémico, pero al no encontrar mejoría con el tratamiento su diagnóstico fue replanteado. En una nueva hospitalización se le identificaron múltiples adenomegalias. Se realizó la biopsia de una de las adenomegalias, la patología fue compatible con LAIT. Se indicó 3 sesiones de quimioterapia, sin embargo, desarrolló falla multiorgánica con desenlace fatal. El LAIT es un reto diagnóstico debido a que puede imitar varias patologías autoinmunes. Es muy importante su sospecha y descarte para iniciar un tratamiento precoz que mejore la sobrevida de los pacientes.

Angioimmunoblastic T-cell lymphoma (LAIT) is a rare non-Hodgkin lymphoma, can mimic autoimmune diseases, and has a poor prognosis. We present the case of a 36-year-old woman with a 3-year illness characterized by fever, arthralgia and weight loss. She was initially diagnosed as systemic lupus erythematosus, but finding no improvement with treatment, her diagnosis was reconsidered. In a new hospitalization, multiple lymph nodes were identified. They performed a biopsy of one of the adenopathies, the pathology was compatible with LAIT. Three chemotherapy sessions were indicated, however, she developed multiple organ failure with a fatal outcome. LAIT is a diagnostic challenge because it can mimic several autoimmune pathologies. Its suspicion and ruling out is very important to initiate early treatment that improves patient survival.

Linfoma gástrico primario de células T, no asociado a HTLV-1, con metástasis cutánea y neumoperitoneo / Non-HTLV-1-associated primary gastric T-cell lymphoma with cutaneous metastasis and pneumoperitoneum

Introducción: Los linfomas gástricos primarios representan menos del 5% de los linfomas no Hodgkin (LNH). La gran mayoría de linfomas gástricos primarios son linfomas de células B de alto grado. Los linfomas gástricos primarios de células T son muy raros y usualmente se asocian a infección por HTLV-1 en regiones endémicas. Material y método. Describimos el caso de una paciente mujer de mediana edad que se presentó con neumoperitoneo debido a ulcera gástrica perforada, síndrome consuntivo y lesiones cutáneas y orales. Resultados. La histopatología e inmunohistoquímica confirmaron linfoma gástrico primario de células T con compromiso cutáneo. La serología para HTLV-1, virus del Epstein-Barr y VIH fueron negativas. Conclusión. La finalidad de este reporte es dar a conocer esta forma de presentación sumamente rara de linfoma gástrico primario.

Background: Primary gastric lymphomas account for less than5%ofnon-Hodgkinlymphomas(NHL).Thevast majority of primary gastric lymphomas are high-grade B-cell lymphomas. Primary gastric T-cell lymphomas are very rare and are usually associated with HTLV-1 infection in endemic regions. We describe Material and methods.the case of a middle-aged female patient who presented with pneumoperitoneum due to a perforated gastric ulcer, wasting syndrome, and skin and oral lesions. . Results: Histopathology and immunohistochemistry confirmed primary gastric T-cell lymphoma with skin involvement. The serologyfor HTLV-1,Epstein-Barr virus and HIVwere negative. The aim of this report is to present Conclusion: this extremely rare presentation of primary gastric lymphoma

Silencioso e negligenciado: o que se sabe após quatro décadas do descobrimento do HTLV-1? / Silent and neglected: what is known after four decades of the discovery of the HTLV-1? / Silencioso y descuidado: ¿qué se sabe después de cuatro décadas del descubrimiento del HTLV-1?

O vírus linfotrópico T humano tipo 1 (HTLV-1) foi o primeiro retrovírus humano descoberto, descrito pela primeira vez há 41 anos. Esse retrovírus está associado ao desenvolvimento de duas doenças graves: a leucemia/linfoma de células T do adulto (ATLL) e a mielopatia associada ao HTLV-1/paraparesia espástica tropical (HAM/TSP). Este trabalho teve como objetivo analisar as atualizações sobre o HTLV-1, destacando os aspectos clínicos, os avanços e as limitações no tratamento e na prevenção da infecção pelo HTLV-1. Para isso, foi realizada uma revisão integrativa, por meio de coleta de dados nas plataformas PubMed, LILACS e SciELO, entre março e abril de 2021. Foram incluídos 61 artigos de diferentes países. O Brasil foi o país com maior número de publicações na área: 12. Os resultados obtidos mostram que existem avanços importantes no que diz respeito ao tratamento e à prevenção da infecção pelo HTLV-1. No entanto, a falta de estudos específicos sobre o vírus, que abordem os aspectos clínicos da infecção, foi um fator limitante para este estudo, o que reforça a necessidade de investimento em novas pesquisas sobre o tema.

The Human T-lymphotropic Virus 1 (HTLV-1) was the first human retrovirus discovered, described for the first time 41 years ago. This retrovirus is associated with the development of two serious diseases: adult T-cell leukemia/lymphoma (ATLL) and tropical spastic paraparesis/HTLV-1-associated myelopathy (HAM/TSP). This study aimed to analyze the updates about HTLV-1, highlighting the clinical aspects, advances, and limitations in the treatment and prevention of HTVL-1 infection. To this end, an integrative review was carried out, with data collection on PubMed, LILACS, and SciELO platforms, between March and April 2021. A total of 61 articles from different countries were included. Brazil was the country with the largest number of publications in the area: 12. The results showed effective advances regarding treating and preventing HTLV-1 infection. However, the lack of specific studies about the virus, which address the clinical aspects of the infection, was a limiting factor for this study, which reinforces the need for investment in new research about this topic.

El virus linfotrópico T tipo 1 humano (HTLV-1) fue el primer retrovirus humano descubierto y se describió por primera vez hace 41 años. Este retrovirus está asociado con el desarrollo de dos enfermedades graves: leucemia/linfoma de células T del adulto (ATLL) e mielopatía asociada a HTLV-1/paraparesia espástica tropical (HAM/TSP). Este estudio tuvo como objetivo analizar las actualizaciones sobre HTLV-1, destacando los aspectos clínicos, los avances y limitaciones en el tratamiento y prevención de la infección por HTLV-1. Para ello, se realizó una revisión integradora, a través de la recolección de datos en las plataformas PubMed, LILACS y SciELO entre marzo y abril de 2021. Se incluyeron 61 artículos de diferentes países. Brasil fue el país con mayor número de publicaciones en el área: 12. Los resultados obtenidos muestran que existen avances efectivos en cuanto al tratamiento y prevención de la infección por HTLV-1. Sin embargo, la falta de estudios específicos sobre el virus que aborden los aspectos clínicos de la infección fue un factor limitante para el presente estudio, lo que refuerza la necesidad de invertir en nuevas investigaciones sobre este virus.

Peripheral T-cell lymphoma not otherwise specified with oral manifestation: a case report / Linfoma periférico de células T no especificado con manifestación oral: reporte de caso

Peripheral T-Cell Lymphoma Not Otherwise Specified it is a rare type of Non-Hodgkin t-cells malignant tumor whose oral manifestations are difficult to diagnose. A case of a 48-year-old male with a hemi-maxillary lesion histological and immunohistochemically compatible with Peripheral T-Cell Lymphoma not otherwise specified is presented. A case of a 48-year-old male with a hemi-maxillary lesion histological and immunohistochemically compatible with Peripheral T-Cell Lymphoma not otherwise specified is presented. The patient treatment consisted of chemotherapy, but after the second cycle, died from immunosuppressive complications. Early stage diagnosis of oral lesions is imperative to avoid aggressive treatment and low overall survival rate of such pathologies.

Introducción: El linfoma periférico de células T no especificado es un tipo raro de tumor maligno no Hodgkin de células T cuyas manifestaciones orales son difíciles de diagnosticar. Se presenta el caso de un varón de 48 años con lesión hemimaxilar histológica e inmunohistoquímicamente compatible con linfoma periférico de células T no especificado. El tratamiento del paciente consistió en quimioterapia, pero después del segundo ciclo, falleció por complicaciones inmunosupresoras. El diagnóstico temprano de las lesiones orales es imperativo para evitar un tratamiento agresivo y la baja tasa de supervivencia.

Leucemia/linfoma T del adulto HTLV1, un desafío para el clínico / Leukemia / Lymphoma T Of The Adult HTLV1, A Challenge For The Clinic

La leucemia / linfoma de células T en adultos (LLTA) es una enfermedad agresiva de células T maduras activadas causada por el virus linfotrópico de células T humano tipo 1 (HTLV-1). ATL tiene un mal pronóstico debido a la quimiorresistencia intrínseca y la inmunosupresión severa. Las formas agresivas de LLTA, aguda y linfoma, se tratan con quimioterapia asociada con agentes antirretrovirales (AZT / IFN). Sin embargo, no han logrado un impacto en la supervivencia, que oscila entre 8 y 10 meses, respectivamente. Los pacientes con formas de LLTA crónicas y latentes tienen un mejor pronóstico, pero la supervivencia a largo plazo también es deficiente, tanto cuando estos pacientes se manejan con una política de espera vigilante o con quimioterapia. Aparentemente, las costuras AZT / IFN benefician a estos pacientes. Mientras tanto, la prevención de la diseminación del HTLV-1 es imprescindible en las políticas de salud pública, tanto por tamizaje del virus en bancos de sangre como a mujeres embarazadas para reducir / evitar la transmisión vertical del virus.

Adult T-cell leukemia/lymphoma (ATL) is an aggressive disease of mature activated T cell caused by human T-cell lymphotropic virus type 1 (HTLV-1). ATL carries a bad prognosis due to intrinsic chemoresistance and severe immunosuppression. The aggressive ATL forms, acute and lymphoma, are treated with chemotherapy associated with antiretroviral agents (AZT/IFN) the acute form. However, they have failed to achieve an impact on survival, that ranges from 8-10 months, respectively. Patients with chronic and smoldering ATL forms, have a better prognosis, but long term survival is poor as well, when these patients are managed with a watchfulwaiting policy or with chemotherapy. Apparently, AZT/IFN seams to benefit these patients. Meanwhile, prevention of dissemination of HTLV-1, is a must in public health policies, performing screening in blood banks and a screening to pregnant women to reduce/avoid vertical transmission of the virus.

Linfoma anaplásico de grandes células relacionado ao implante mamário: revisão sistemática da literatura / Breast implant-associated anaplastic large-cell lymphoma: a systematic literature review

O linfoma anaplásico de grandes células associado ao implante de mama (Breast Implant Associated Anaplastic Large Cell Lymphoma - BIA-ALCL) é uma doença maligna recentemente descoberta, rara e possivelmente associada aos implantes mamários texturizados. Essa revisão da literatura teve como objetivo trazer novas atualizações acerca da epidemiologia, fisiopatologia e fatores de risco para desenvolvimento do BIAALCL. Foi realizado o levantamento de dados do período de dezembro de 2018 a fevereiro de 2019, através das bases de dados PUBMED, LILACS e Scielo sendo selecionados 10 artigos publicados entre 2016 e 2018. Foi encontrada uma incidência variando entre 2,8:100.000 a 1:3 milhões de pacientes com implantes mamários. Os dados coletados corroboram para a teoria de que não há uma relação direta de causa e efeito entre os implantes mamários, mormente os texturizados, e o desenvolvimento do BIA-ALCL, podendo esses ser considerados somente como fatores de risco e não agentes causadores. A teoria fisiopatológica mais aceita é a de que os implantes mamários com maior área de superfície levariam a formação de maior biofilme por maior adesão bacteriana gerando inflamação crônica mais proeminente, levando ao gatilho para a transformação maligna das células T. As informações explicitadas nessa revisão devem auxiliar na ampliação de estudos acerca da doença e criação de políticas públicas para a prevenção e diagnóstico precoce de tal enfermidade. Pelos dados encontrados há necessidade de que cirurgiões plásticos realizem acompanhamento mais próximo de seus pacientes, assim como orientem os pacientes antes das cirurgias sobre a existência da doença.

Breast implant-associated anaplastic large-cell lymphoma (BIA-ALCL) is a newly discovered and rare cancer possibly associated with textured breast implants. This literature review investigates its epidemiology, pathophysiology, and risk factors. PubMed, LILACS, and SciELO databases were searched from December 2018 to February 2019, and 10 articles published between 2016 and 2018 were selected. The incidence of BIA-ALCL ranged from 2.8:100,000 to 1:3 million breast implants. The obtained data corroborate the hypothesis that there is no direct cause and effect relationship between breast implants, especially textured implants, and BIA-ALCL, and these implants can be considered risk factors but not causative factors. The most accepted hypothesis on disease pathophysiology is that breast implants with larger surface areas may promote bacterial adhesion and biofilm formation, leading to severe chronic inflammation, triggering the malignant transformation of T cells. This review provides knowledge on BIA-ALCL and helps develop and implement public policies for disease prevention and timely diagnosis. The data highlight that long-term follow up is necessary and that surgeons should advise patients of the potential risk of developing BIA-ALCL before performing the implant surgery.

Anaplastic lymphoma mimicking breast carcinoma / Linfoma anaplásico mimetizando carcinoma mamário

This case describes an uncommon presentation of ALK-negative anaplastic large T-cell lymphoma with breast infiltration, mimicking triple-negative carcinoma. The incidence of ALK-negative anaplastic large T-cell lymphoma usually occurs in adults in their fifth and sixth decade of life and can affect lymph nodes and extranodal sites, including skin, soft tissue, and gastrointestinal tract. The non-Hodgkin's lymphoma of the breast is uncommon, accounting for 0.04 to 0.05% of all malignant breast tumors. Diagnosis of ALK-negative anaplastic large T-cell lymphoma is challenging both to physicians and pathologists. Based on the complete medical history, clinical and imaging exams and histopathological evaluation of the lesion site biopsy, it is possible to establish an adequate diagnosis. The case describes a woman aged 37 years with palpable nodules in the left breast as well as erythematous lesions on the right leg. The analysis of the breast nodules biopsy shows that they mimic triple-negative carcinoma. However, only with immunohistochemical examination was it possible to verify the expression of the CD30 antigen, and only after a complete systemic evaluation, the diagnosis of ALK-negative anaplastic large T-cell lymphoma was performed. Misdiagnosis can lead to inadequate therapy and result in disease progression or unnecessary damages to the patient.

Este caso descreve uma incomum apresentação de linfoma anaplásico de grandes células T ALK negativo com infiltrado mamário, mimetizando carcinoma triplo negativo. A incidência do linfoma anaplásico de grandes células T ALK negativo, ocorre comumente em adultos na quinta e sexta década de vida e pode acometer linfonodos e locais extranodais, incluindo pele, tecido mole e trato gastrointestinal. O linfoma não-Hodgkin da mama é incomum, compondo 0,04 a 0,05% de todos os tumores de mama malignos. O diagnóstico de linfoma anaplásico de grandes células T ALK negativo é desafiador tanto para clínicos como para patologistas. O estabelecimento de um diagnóstico adequado é possível com base em histórico médico completo, exames clínicos e de imagem e avaliação histopatológica da biópsia do local da lesão. O caso relata uma mulher de 37 anos com nódulos palpáveis na mama esquerda em conjunto com lesões eritematosas na perna direita. Ao se analisar a biópsia dos nódulos da mama, esses mimetizavam carcinoma triplo negativo, no entanto, somente com exame imunohistoquímico foi possível verificar a expressão do antígeno CD30, e, apenas após uma avaliação sistêmica completa, foi realizado o diagnóstico de linfoma anaplásico de grandes células T ALK negativo. O diagnóstico equivocado pode acarretar terapia inadequada e resultar em progressão da doença ou em danos desnecessários ao paciente.

Leucemia/Linfoma de células T del adulto y criptococosis sistémica: Reporte de dos casos en un hospital peruano / Adult T-cell leukemia/lymphoma and systemic cryptococcosis: report of two cases in a Peruvian hospital

La leucemia/linfoma de células T adulto (LLCTA) es una neoplasia linfoide causada por el virus linfotrópico humano de células T tipo 1 (HTLV-1), retrovirus endémico en nuestro país. Tiene una presentación heterogénea, con variedades de pobre pronóstico e infecciones oportunistas que lo ensombrecen aún más. Se presentan dos casos de pacientes con variedades agresivas de la enfermedad (aguda y linfomatosa) complicadas con criptococosis sistémica. Reforzamos la necesidad de un enfoque diagnóstico completo y un monitoreo continuo de agentes oportunistas en el paciente con LLCTA adaptado a nuestro contexto epidemiológico

Adult T-cell leukemia/lymphoma (ATLL) is a lymphoid neoplasm caused by the human T-cell lymphotropic virus type 1 (HTLV-1), an endemic retrovirus in our country. It has a heterogeneous presentation, with sub types of poor prognosis, and opportunistic infections that worsen the prognosis. We present two cases with aggressive varieties of the disease (acute and lymphomatous) complicated by systemic cryptococcosis. It is necessary to conduct a complete diagnostic approach and continuous monitoring of opportunistic agents in patients with ATL adapted to our epidemiological context.

Falla hepática aguda fulminante como manifestación de leucemia de células T del adulto en paciente con infección por HTLV-1 / Acute Liver Failure as Presentation of Adult T-cell Leukemia in an HTLV-1 Patient

La falla hepática aguda es el desarrollo de injuria hepática severa con deterioro de la función de síntesis y encefalopatía. Dentro de la variedad de causas, las infiltraciones neoplásicas representan menos del 0,5%. Presentamos el caso de un paciente varón HTLV1 positivo que debuta con una clínica de hepatitis aguda, siendo posteriormente diagnosticado con diseminación de linfoma/leucemia de células T del adulto. Desafortunadamente el paciente presentó deterioro rápido y progresivo de la función hepática, falleciendo a los pocos días de la hospitalización

Acute liver failure is the development of severe hepatic injury with deterioration of liver synthesis function and encephalopathy. Among all the variety of causes, neoplastic infiltration represents less than 0.5%. We present the case of a male patient with a past medical history of HTLV-1 infection, who reports symptoms of acute hepatitis, being diagnosed with Adult T-cell leukemia/ lymphoma. Unfortunately, the patient had a rapid deterioration and passed away a few days after admission

Linfoma anaplásico de células grandes CD30+ y ALK+ con manifestación oral: revisión de literatura y reporte de caso / Anaplastic large cell lymphoma CD30+ and ALK+ with oral manifestation: review of the literature and case report

Introducción: Los linfomas son neoplasias del sistema linfoide clasificadas en Hodgkin y No-Hodgkin. Los linfomas de cabeza y cuello se originan en tejido linfoide regional, pero también en otros sitios como encía, paladar, etc. Objetivo: Describir el Linfoma Anaplásico de Células Grandes (LACG) como un subgrupo de linfomas No-Hodkin de células T, positivo o negativo para la expresión de las proteínas CD30 y AKL, el cual puede afectar numerosas partes del cuerpo incluyendo la cavidad oral. Se reporta un caso de LACG de células T CD30+ALK+. Diseño: Reporte de caso. Metodología: Se realizó una búsqueda sistemática en PubMed, MEDLINE, EMBASE, LILACS y Ovid. Se seleccionaron los artículos que reportaron casos de Linfoma No-Hodkin de células T con manifestaciones orales, limitándose al idioma Inglés. Reportamos el caso de una paciente de 9 años con LACG de células T, CD30+ALK+ con manifestaciones orales y sistémicas. Resultados: De los artículos obtenidos ninguno reporta casos de LACG de células T CD30+ ALK+ con manifestaciones orales, por la cual no fue posible realizar el análisis sistemático. Discusión: El LACG representa 2-3% de los Linfomas No-Hodkin, se divide en cutáneo primario y sistémico (ALK+/ALK-). El ALK+ también puede afectar sitios extranodales. Los pacientes a menudo presentan síntomas sistémicos, como fiebre y sudoración nocturna. Los casos de LACG sistémico primario ALK+ suelen responder bien a la quimioterapia y tienen mejor pronóstico que los casos ALK-. Conclusiones: El Linfoma anaplásico de células grandes de células T, CD30+ALK+ es una entidad poco frecuente en la cavidad oral.

Introduction: Lymphomas are neoplasms of the lymphoid system, they are classified as Hodgkin and Non-Hodgkin. Head and neck lymphomas are originated in regional lymphoid tissue, but also in other sites such as gums, lips, palate, etc. Objective: To describe the Anaplastic Large Cell Lymphoma (ALCL) as a subgroup of T-cell non-Hodgkin's lymphoma, positive or negative for expression of CD30+ and AKL+proteins, which can affect many parts of the body including tissue of oral cavity. Finally, it is pretended to report a case of ALCL T-cell, CD30+ AKL+. Design: Case report. Materials and methods: A systematic search was performed using electronic databases such as PUBMED, MEDLINE, EMBASE, LILACS and Ovid. Those papers which reported cases of T-cell NHL with oral manifestations were selected for further evaluation. This search was limited to english language. Additionally, the case of an ALCL T-cell, CD30+AKL+ with oral and systemic manifestations in a 9-year-old girl treated at the Fundación Hospital de la Misericordia is reported. Results: Of the articles obtained none report cases of ALCL T-cell, CD30+AKL+ with oral manifestations, so it is not possible to perform a systematic review of them. Discussion: ALCL is classified as primary cutaneous and systemic (ALK + and ALK-). The ALCL represents 2-3% of all NHL. The ALK+ ALCL can also affect extranodal sites. Patients often have systemic symptoms, such as fever and night sweats. Cases of ALK+ primary systemic ALCL usually respond well to chemotherapy and have a better prognosis compared to ALK- cases which have a less favorable prognosis with unpredictable clinical behavior. Conclusions: ALCL T-cell, CD30+AKL+ is an entity with presentation rare in the oral cavity.

Linfoma duodenal asociado a infección por Strongyloides stercoralis. Dos manifestaciones de la infección por HTLV-1 / Duodenal Lynphoma associated to Strongyloides stercoralis infection. Two types of HTLV-1 infection

La infección por el virus linfotrópico de células T humanas tipo 1 (HTLV-1), es causante de la leucemia/linfoma de células T del Adulto (ATLL), siendo la afectación duodenal poco usual. La coinfección de HTLV-1 con Strongyloides stercolaris es común en los pacientes con HTLV- 1 debido a la inadecuada respuesta TH2 que presentan estos sujetos. Describimos a una paciente mujer de 48 años de edad, natural y procedente de la selva del Perú con historia familiar de infección por HTLV- 1 quien acude con diarrea crónica y baja de peso. Se le diagnosticó infección por HTLV-1 así como ATLL duodenal y estrongiloidiasis. Se inició tratamiento con ivermectina y quimioterapia, siendo estabilizada y dada de alta. Reportamos el presente caso debido a la poca frecuencia de coexistencia en duodeno de ATLL y estrongiloidiasis.

Infection by the Human T- Lymphotropic virus I (HTLV-1) causes Adult T cell Leukemia-lymphoma (ATLL), being the duodenal involvement rare. Commonly, patients co-infected with HTLV-1 and Strongyloides stercoralis are seen due to the lack of TH2 response found on these patients. We describe a 48-year-old woman, from the jungle of Peru, with a family history of HTLV-1 infection, who presented with a History of chronic diarrhea and weight loss. HTLV-1 infection with ATLL and strongyloidiasis were diagnosed. Ivermectin treatment and chemotherapy were initiated, being stabilized, and discharged. We report this case because of the unusual coexistence in the duodenum of ATLL and strongyloidiasis.

Adult T-cell leukemia/lymphoma / Leucemia/linfoma de células T do adulto

Summary Adult T-cell leukemia/lymphoma (ATL) is a malignancy of mature CD4+ T-cells caused by human T-cell lymphotropic virus type 1 (HTLV-1). Twenty million people are believed to be infected throughout the world, mostly in Japan, Africa, the Caribbean, and South America, particularly in Brazil and Peru. ATL affects about 5% of infected individuals and is classified in the following clinical forms: acute, lymphoma, primary cutaneous tumoral, chronic (favorable and unfavorable), and smoldering (leukemic and non-leukemic). Although it is considered an aggressive disease, there are cases with a long progression. We emphasize the importance of clinical classification as an indispensable element for evaluating prognosis and appropriate therapeutic approach. Since several cases have been published in Brazil and this disease is still poorly known, we decided to make a review paper for dissemination of clinical, hematological and pathological aspects, diagnosis, and therapy. The best way to reduce the occurrence of ATL would be halting the transmission of the virus through breastfeeding.

Resumo A leucemia/linfoma de células T do adulto (LLcTA) é uma neoplasia de células T maduras CD4+ causada pelo vírus linfotrópico para células T humanas tipo 1 (HTLV-1). Acredita-se que existem cerca de 20 milhões de pessoas infectadas em todo o mundo, principalmente no Japão, na África, no Caribe e na América do Sul, particularmen te no Brasil e no Peru. A LLcTA acomete cerca de 5% dos indivíduos infectados e classifica-se nas seguintes formas clínicas: aguda, linfomatosa, tumoral primária de pele, crônica (favorável e desfavorável) e indolente (leucêmica e não leucêmica). Embora seja considerada uma doença agressiva, há casos com longa evolução. Salientamos a importância da classificação clínica como elemento im prescindível para avaliação do prognóstico e conduta terapêutica adequada. Como já foram publicados vários casos no Brasil e essa doença ainda é pouco conhecida, decidimos fazer um trabalho de revisão para divulgar os seus aspectos clínicos, hematológicos, anatomopatológi cos, diagnósticos e terapêuticos. O melhor meio de redu zir a ocorrência de LLcTA seria sustando a transmissão vertical do vírus pela amamentação.

Linfoma de células t angioimunoblástico em paciente idoso e com diagnóstico prévio de mononucleose infecciosa: um desafio para o tratamento oncológico ? relato de caso / Angioimmunoblastic T-cell lymphoma in elderly patient with a previous diagnosis of infectious mononucleosis: a challenge for cancer treatment ? case report

O linfoma de células T angioimunoblástico (AITL) é subtipo raro de linfoma de células T periférico que representa cerca de 2% dos linfomas não Hodgkin (LNH). Acomete predominantemente homens, na sexta e sétima décadas de vida e se apresenta como doença sistêmica. É agressivo no comportamento e responde mal à quimioterapia, com tratamento padrão ainda não definido. Este relato objetiva apresentar o prognósticodesfavorável nos pacientes com AITL e a falta de consolidação de seu tratamento. Os fatores que podem estar associados à má-resposta são: idoso, estágios avançados e sintomatologia constitucional.

Angioimmunoblastic T-cell lymphoma (AITL) is a rare subtype of peripheral T-cell lymphoma that represents about 2% of non-Hodgkin?s lymphomas (LNH). It affects mainly men, in the sixth and seventh decades of life, and appears as a systemic disease. It has an aggressive behavior and responds poorly to chemotherapy, still with undefined standard treatment. This report aims to provide the poor prognosis in patients with AITLand the lack of treatment consolidation. The factors that may be associated with the poor response are being elderly, advanced stages, and constitutional symptoms.

Linfoma T intestinal monomórfico CD56+. Primer caso informado en Colombia y revisión de la literatura / Monomorphic Intestinal T-Cell Lymphoma. First reported case in Colombia and review of the literature

Se informa el caso de una mujer de 80 años con hallazgo incidental de masa abdominal en mesogastrio. Se le realiza resección de intestino delgado por sospecha de absceso con síndrome adherencial. El estudio patológico de la pieza quirúrgica demostró compromiso por linfoma no Hodgkin T monomórfico CD56+. Los linfomas T del tubo digestivo son inusuales; corresponden al 5% de las neoplasias linfoides de esta localización. El linfoma intestinal T monomórfico CD56+ corresponde a 10-20% de los linfomas T intestinales. Se presenta en regiones donde la enfermedad celiaca es infrecuente y no se asocia a enteropatía. Su curso es agresivo y habitualmente se manifiesta como una emergencia quirúrgica por obstrucción o perforación intestinal.

80 year old woman with incidental finding of abdominal mass in midgut, in which small bowel resection is performed for suspected abscess associated to surgical adhesions. Pathological examination of the surgical specimen showed a monomorphic CD56+ T cell lymphoma. T-cell lymphomas of the digestive tract are rare, accounting for 5% of all lymphoid neoplasms in this location. Monomorphic CD56+ intestinal T cell lymphoma corresponds to 10 - 20% of T cell intestinal lymphomas. This tumor occurs in regions where celiac disease is rare and it is not associated with enteropathy. Its course is aggressive and usually manifests as a surgical emergency by intestinal obstruction or perforation.