Compromiso secundario de glándulas mamarias por linfoma folicular / Secondary involvement of breasts by follicular lymphoma

Resumen Las lesiones metastásicas que comprometen la glándula mamaria son excepcionales, ocupando las neoplasias hematolinfoides el segundo lugar en orden de frecuencia en series de casos reporta dos en la literatura con una prevalencia de 0.04% a 1.6% en relación a todos los tumores malignos de la mama, alcanzando una incidencia anual de 0.07%, los cuales corresponden principalmente a linfomas secundarios. El 80% de estos son linfomas B difusos de células grandes, seguido de linfoma folicular y linfoma de la zona marginal. Presentamos una mujer de 60 años con diagnóstico de linfoma folicular que comenzó con una masa perirrenal derecha y ganglios linfáticos ipsilaterales retroperitoneales e inguinales, quien, durante su tratamiento, presentó avance en el estadio clínico con compromiso secundario inusual de ambas glándulas mamarias por esta neoplasia hematolinfoide. Se evaluó el comportamiento biológico de esta enfermedad para comprender los mecanismos fisiopatológicos, mediante el análisis de factores clínicos, histológicos y pronósticos, permitiendo la estadificación definitiva, la cual fue determinante para la elección de la terapia individualizada acorde a las guías de práctica clínica basada en la evidencia científica, impactando positivamente en la evolución médica de la paciente.

Abstract Metastatic lesions involving the breast are exceptional; hematolymphoid neoplasias rank second as per their frequency in case series reported in the literature with a prevalence of 0.04% to 1.6% when consider ing all malignant breast tumors and reaching an annual incidence of 0.07%, mainly accounted for by secondary lymphomas. Eighty percent of them are diffuse, large B cells lymphomas (DLBCL), followed by follicular lymphoma and marginal zone lymphoma. This case is about a 60 year-old woman with a diagnosis of follicular lymphoma, who presented with a right perirenal mass and ipsilateral retroperitoneal and inguinal lymph nodes, whose clinical status progressed during the treatment with unusual secondary involvement of both breasts by hematolymphoid neoplasia. The biological behavior of the condition was evaluated to understand the pathophysiological mecha nisms; this was done analyzing clinical, histologic and prognostic factors that led to a definitive staging, which was key to select the individualized therapy following the clinical practice guidelines based on scientific evidence, with a positive impact on the patient's medical progress.

Secondary involvement of breasts by follicular lymphoma / Compromiso secundario de glándulas mamarias por linfoma folicular

Abstract Metastatic lesions involving the breast are exceptional; hematolymphoid neoplasias rank second as per their frequency in case series reported in the literature with a prevalence of 0.04% to 1.6% when considering all malignant breast tumors and reaching an annual incidence of 0.07%, mainly accounted for by secondary lymphomas. Eighty percent of them are diffuse, large B cells lymphomas (DLBCL), followed by follicular lymphoma and marginal zone lymphoma. This case is about a 60 year-old woman with a diagnosis of follicular lymphoma, who presented with a right perirenal mass and ipsilateral retroperitoneal and inguinal lymph nodes, whose clinical status progressed during the treatment with unusual secondary involvement of both breasts by hematolymphoid neoplasia. The biological behavior of the condition was evaluated to understand the pathophysiological mechanisms; this was done analyzing clinical, histologic and prognostic factors that led to a definitive staging, which was key to select the individualized therapy following the clinical practice guidelines based on scientific evidence, with a positive impact on the patient's medical progress.

Resumen Las lesiones metastásicas que comprometen la glándula mamaria son excepcionales, ocupando las neoplasias hematolinfoides el segundo lugar en orden de frecuencia en series de casos reportados en la literatura con una prevalencia de 0.04% a 1.6% en relación a todos los tumores malignos de la mama, alcanzando una incidencia anual de 0.07%, los cuales corresponden principalmente a linfomas secundarios. El 80% de estos son linfomas B difusos de células grandes, seguido de linfoma folicular y linfoma de la zona marginal. Presentamos una mujer de 60 años con diagnóstico de linfoma folicular que comenzó con una masa perirrenal derecha y ganglios linfáticos ipsilaterales retroperitoneales e inguinales, quien, durante su tratamiento, presentó avance en el estadio clínico con compromiso secundario inusual de ambas glándulas mamarias por esta neoplasia hematolinfoide. Se evaluó el comportamiento biológico de esta enfermedad para comprender los mecanismos fisiopatológicos, mediante el análisis de factores clínicos, histológicos y pronósticos, permitiendo la estadificación definitiva, la cual fue determinante para la elección de la terapia individualizada acorde a las guías de práctica clínica basada en la evidencia científica, impactando positivamente en la evolución médica de la paciente.

Recaída de linfoma folicular de alto grado en tiroides

El linfoma folicular de tiroides es una entidad rara que representa menos del 2% de los linfomas extranodales y cuyo principal factor de riesgo es la tiroiditis de Hashimoto. Suele afectar con mayor predisposición a pacientes adultos mayores, en los que con frecuencia se presenta como una masa de crecimiento rápido con compromiso de las estructuras adyacentes en el cuello. Sin embargo, dada su rara incidencia, esta patología debe ser sospechada por el clínico para un diagnóstico temprano oportuno. Aunque en la actualidad no existen guías de manejo estandarizadas para esta entidad, acostumbra tener buena respuesta a radioterapia y quimioterapia. El presente caso describe la historia de un paciente con una recaída de linfoma no Hodgkin de tipo folicular de alto grado localizado en tiroides que se presentó a urgencias con disfonía, parálisis bilateral de cuerdas vocales, cambio en el tono de la voz y disfagia, con el antecedente de un linfoma folicular de alto grado en localización retroperitoneal y en remisión luego de 8 ciclos de quimioterapia con rituximab, ciclofosfamida, doxorubicina, vincristina y prednisona, el cual presentó citorreducción luego del tratamiento con quimioterapia con el protocolo de rituximab, dexametasona, citarabina y cisplatino.

Follicular thyroid lymphoma is a rare entity that represents less than 2% of extranodal lymphomas; and whose main risk factor is Hashimoto's thyroiditis. This tends to affect older adult patients with greater predisposition, where it usually presents as a rapidly growing mass with compromise of the adjacent structures in the neck. However, given its rare incidence, this pathology should be suspected by the clinician for an early diagnosis. Although there are currently no standardized management guidelines for this entity, it usually responds well to radiotherapy and chemotherapy. The present case describes the history of a patient with a relapse of high-grade follicular-type NHL located in the thyroid who presented to the emergency room with dysphonia, bilateral paralysis of the vocal cords, change in tone of voice and dysphagia, with the history of high-grade follicular lymphoma in retroperitoneal location in remission after 8 cycles of chemotherapy with Rituximab, Cyclophosphamide, Doxorubicin, Vincristine and Prednisone, which presented cytoreduction after treatment with chemotherapy with the protocol Rituximab, Dexamethasone, Cytarabine and Cisplatin.

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

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

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

Linfoma no Hodgkin: primera y segunda neoplasia o, transformación histológica: dificultad diagnóstica: reporte de un caso / Non-Hodgkin's lymphoma: first and second neoplasia or, histological transformation: diagnostic difficulty: a case report

El LF es una enfermedad heterogénea con muchos diferentes subgrupos, en términos de edad de inicio, órganos involucrados (especialmente sitios extranodales) y anormalidades genéticas. Con grandes variaciones en el curso clínico, algunas veces muy indolente o no; puede mostrar transformación a linfomas de alto grado, se han reportado transformaciones a Linfoma de Células Grandes B Difusas (siendo este el más común), de Burkitt, Leucemia/Linfoma, Linfoma Linfoblástico (reportado en raras ocasiones), con un curso clínico agresivo. En el 2008 la OMS estableció 3 nuevas variantes: 1) LF Pediátrico, 2) LF Primario Intestinal y 3) LF in situ. Patólogos y clínicos debemos considerar estas patologías cuando se decide el diagnóstico y el tratamiento. La transformación histológica se refiere al evento biológico que lleva al desarrollo de un Linfoma No Hodgkin agresivo de alto grado en pacientes con un LF subyacente. El Linfoma Folicular (LF) pediátrico es una neoplasia excesivamente rara, que difiere del LF del adulto.

FL is a heterogeneous disease with many different subgroups, in terms of age of onset, organs involved (especially extranodal sites) and genetic abnormalities. With great variations in the clinical course, sometimes very indolent or not; can show transformation to high-grade lymphomas, transformations to Diffuse Large B Cell Lymphoma (this being the most common), Burkitt's, Leukemia / Lymphoma, Lymphoblastic. Lymphoma (reported rarely), with an aggressive clinical course have been reported. In 2008 the WHO established 3 new variants: 1) Pediatric FL, 2) Intestinal Primary FL and 3) in situ FL. Pathologists and clinicians must consider these pathologies when deciding on diagnosis and treatment. Histological transformation refers to the biological event that leads to the development of high-grade aggressive Non-Hodgkin's Lymphoma in patients with an underlying FL. Pediatric Follicular Lymphoma (FL) is an excessively rare neoplasm, which differs from adult FL.

Linfomas en la era COVID-19 / Lymphoma and COVID-19

Resumen El coronavirus 2 del síndrome respiratorio agudo grave (SARS-CoV-2) y su traducción clínica, la enfermedad por coronavirus 2019 (COVID-19), representan una enfermedad con manifestaciones respiratorias potencialmente fatales. Actualmente existen aproximadamente 12,700,000 personas afectadas por esta virus, el cual ha ocasionado 561,517 muertes en el mundo. Los pacientes con diagnóstico de linfoma, al igual que otros pacientes con cáncer activo, presentan compromiso inmunitario, ya sea por su propia patología o debido al tratamiento que reciben, por lo que son especialmente susceptibles a desarrollar cuadros graves de COVID-19. La transmisión comunitaria del SARS-CoV-2 dificulta el acceso al sistema de salud y, por ende, el seguimiento estricto que requieren lo pacientes bajo tratamiento oncológico. En la etapa en la que nos encontramos actualmente, la transmisión global de la infección por SARS-CoV-2 continúa en ascenso, por lo que un control epidemiológico cercano es poco probable. Ante este contexto, surge la necesidad de establecer guías de tratamiento de pacientes con neoplasias hematológicas.

Abstract The SARS-CoV-2 virus and its clinical translation COVID-19 represent a disease with potentially fatal respiratory manifestations. Currently, there are approximately 12,700,000 people affected by this virus, which has caused 561,517 deaths worldwide. Patients with a diagnosis of lymphoma, like other patients with active cancer, have immune compromise either due to their own pathology or due to the treatment they receive, making them especially susceptible to developing severe cases of COVID-19. The community transmission of SARS-CoV-2 hinders access to the health system and, therefore, the strict monitoring required by patients undergoing cancer treatment. At the stage we are currently in, global transmission of SARS-CoV-2 infection continues to rise, making close epidemiological control unlikely. In this context, the need arises to establish guidelines for the treatment of patients with hematological malignancies.

Fludarabina en el tratamiento del linfoma no Hodgkin folicular / Fludarabine in the treatment of follicular non-Hodgkin lymphoma

RESUMEN Fundamento: el linfoma no Hodgkin folicular ocupa el segundo lugar entre los linfomas no Hodgkin de estirpe B, se caracterizan por un patrón de remisiones y recaídas continuas. La fludarabina constituye una opción de tratamiento del mismo al lograr una rápida remisión, con disminución del porciento de recaída. Objetivo: evaluar la fludarabina como tratamiento de rescate del linfoma no Hodgkin folicular. Métodos: se realizó un estudio observacional analítico de cohortes, longitudinal prospectivo concurrente. El universo de estudio lo conformaron los pacientes que acudieron a la consulta de Hematología con diagnóstico de linfoma no Hodgkin folicular y quedó constituido por 63 pacientes, de los cuales 21 seleccionados mediante muestreo no probabilístico intencionado que recibieron tratamiento con fludarabina, conformaron cohorte de expuestos, que se compararon con 42 pacientes con similares características que recibieron otros esquemas de poliquimioterapia que no incluyó la fludarabina y que constituyó la cohorte de no expuestos. Resultados: fue mayor el número de pacientes con más de 50 años y del sexo masculino, perteneciente al estadio IV de Cotswold y los que tuvieron un riesgo de recaída de bajo - intermedio. El tiempo de sobrevida libre de enfermedad fue mayor en los tratados con fludarabina quienes presentaron menos complicaciones. Conclusiones: en los pacientes tratados con la fludarabina como tratamiento de rescate del linfoma folicular el tiempo de sobrevida libre de la enfermedad fue mayor y se presentaron menos complicaciones, lo que demuestra su utilidad como tratamiento de primeria línea en ausencia del rituximab.

ABSTRACT Background: follicular non-Hodgkin's lymphoma occupies the second place among non-Hodgkin's lymphomas of line B, they are characterized by a pattern of continuous remissions and relapses. Fludarabine constitutes a treatment option for the same as it achieves a rapid remission, with a decrease in the percentage of relapse. Objective: to evaluate fludarabine as a rescue treatment for follicular non-Hodgkin lymphoma. Methods: a concurrent prospective longitudinal, analytical, observational cohort study was conducted. The study universe was made up of patients who attended the Hematology consultation with a diagnosis of follicular non-Hodgkin's lymphoma and consisted of 63 patients, of which 21 were selected by intentional non-probabilistic sampling who received treatment with fludarabine, made up a cohort of exposed, these were compared with 42 patients with similar characteristics who received other poly-chemotherapy regimens that did not include fludarabine and they constituted the unexposed cohort. Results: the number of male patients over 50 years of age, belonging to Cotswold stage IV, and those with a low-intermediate risk of relapse was greater. The disease-free survival time was longer in those treated with fludarabine, who had fewer complications. Conclusions: in patients treated with fludarabine as rescue treatment for follicular lymphoma, the disease-free survival time was longer and there were fewer complications, which show its usefulness as a first-line treatment in the absence of rituximab.

Linfoma folicular tipo duodenal: reporte de caso y revisión de la literatura

El linfoma folicular de tipo duodenal es una neoplasia maligna rara que representa menos del 4% de los casos primarios de linfoma no Hodgkin del tracto gastrointestinal y es una entidad nueva que se describió recientemente en la nueva actualización de la OMS 2016. Actualmente faltan datos sobre los resultados a largo plazo, y por esa razón, no se ha establecido un consenso sobre el manejo de esta enfermedad y su tratamiento. Presentamos un caso de una paciente de 57 años diagnosticada con linfoma folicular de tipo duodenal grado 3a que fue tratado con R-CHOP. El objetivo de este estudio es agregar más datos para una mayor caracterización de la entidad y así seleccionar el mejor manejo para cada caso.

Duodenal type follicular lymphoma is a rare malignancy accounting for less than 4% of primary non-Hodgkin lymphomas of the gastrointestinal tract and it is a new entity that was recently described in the new update WHO 2016. Data regarding long-term outcome are currently lacking, and for that reason, a consensus on the management of this disease has not been established and treatment. We report a case of a 57-year-old female patient diagnosed with duodenal- type follicular lymphoma grade 3a who was treated with R-CHOP. The aim of this study is to add more data for a greater characterization of the entity and thus select the best management for each case.

Criterios diagnósticos y nuevas opciones terapéuticas para los pacientes con diagnóstico de linfoma no Hodgkin / Diagnostic criteria and new therapeutic options for patients diagnosed with non-Hodgkin lymphoma

RESUMEN Fundamento: las hemopatías malignas día a día aumentan su incidencia y proliferación dentro de la población, entre ellas el síndrome linfoproliferativo ocupa un lugar importante, es un grupo de trastornos de origen clonal, que afecta a las células linfoides, con características heterogéneas desde el punto de vista clínico, histológico y molecular. Objetivo: agrupar diferentes criterios diagnósticos y divulgar nuevas opciones terapéuticas para los pacientes con diagnóstico de linfoma no Hodgkin. Métodos: se realizó una búsqueda bibliográfica en las plataformas de acceso a bases de datos de la red de información de salud cubana Infomed, dentro de ellas en EBSCOhost, PubMed/Medline y SciELO. Se utilizaron los siguientes descriptores: síndromes linfoproliferativos, linfomas, linfoma no Hodgkin. Resultados: se aborda la evolución histórica de los síndromes linfoproliferativos desde su descripción inicial hasta el momento actual, así como las diferentes clasificaciones, los tratamientos convencionales utilizados y una panorámica de las investigaciones que se realizan en busca de nuevos tratamientos. Conclusiones: la clasificación de los linfomas no Hodgkin ha cambiado a lo largo del tiempo, la extensión de la enfermedad tiene un papel fundamental en la selección del tratamiento y posible sobrevida del paciente. Existe una amplia variedad de prometedores agentes en desarrollo disponibles para el tratamiento del linfoma folicular.

ABSTRACT Background: malignant haemopathies increase their incidence and proliferation within the population, among them the lymphoproliferative syndrome occupies an important place, it is a group of disorders of clonal origin, which affects the lymphoid cells, with heterogeneous characteristics from the point of clinical, histological and molecular view. Objective: to group different diagnostic criteria and to disclose new therapeutic options for patients diagnosed with non-Hodgkin's lymphoma. Methods: a bibliographic search was carried out in the access platforms to databases of the Infomed Cuban health information network, within them in EBSCOhost, PubMed / Medline and SciELO. The following descriptors were used: lymphoproliferative syndromes, lymphomas, non-Hodgkin's lymphoma. Results: the historical evolution of the lymphoproliferative syndromes is addressed from its initial description to the present time, as well as the different classifications, the conventional treatments used and an overview of the research carried out in search of new treatments. Conclusions: the classification of non-Hodgkin lymphomas has changed over time; the extent of the disease has a fundamental role in the selection of treatment and possible survival of the patient. There is a wide variety of promising developing agents available for the treatment of follicular lymphoma.

Aspectos epidemiológicos, clínicos e anatomopatológicos do linfoma folicular em cães / Epidemiological, clinical and pathological aspects of follicular lymphoma in dogs

Linfomas foliculares são uma rara forma de distúrbio linfoproliferativo descrita em medicina veterinária. Juntamente com a não reconhecida ocorrência dos linfomas de Hodgkin em cães, essa é a maior diferença acerca de linfoma entre humanos e cães. O objetivo deste artigo é descrever os achados epidemiológicos, clínicos e anatomopatológicos vistos em cinco cães com linfoma folicular. Destes, dois eram machos (40%) e três eram fêmeas (60%). A idade dos cães afetados variou de 11 a 13 anos. Quatro dos cinco (80%) cães eram de raça pura e um (20%) não tinha raça definida. Todos os cães apresentaram linfadenomegalia generalizada e esplenomegalia, o que incluiu os casos como linfoma multicêntrico. Na necropsia, os linfonodos e o baço demonstraram um padrão nodular à superfície de corte, caracterizado por dezenas a centenas de nódulos brancos, multifocais ou coalescentes e de tamanhos variáveis. Na superfície natural do baço, frequentemente (4/5, 80%), havia miríades de pontos brancos, multifocais ou coalescentes, de tamanhos variáveis. Na histopatologia, os tumores foram confirmados como linfomas foliculares. Todos os casos eram Grau III, sendo dois (40%) incluídos como IIIa e outros três (60%) como IIIb. Em um caso (1/5, 20%), o linfoma folicular foi considerado como IIIb variante de pequenos centroblastos semelhantes aos linfócitos neoplásicos vistos no linfoma de Burkitt. Os linfomas foram validados como tendo origem em células B através da imuno-histoquímica, utilizando anticorpos anti-CD20. Os casos de linfomas foliculares descritos comportaram-se de forma agressiva e levaram os pacientes à morte.(AU)

Follicular lymphomas are a rare form of lymphoproliferative disorder described in veterinary medicine. Together with the probable non-existence of Hodgkin's lymphomas in dogs, this is the biggest difference about lymphoma between humans and dogs. The aim of this article is to describe the epidemiological, clinical and anatomopathological findings observed in five dogs with follicular lymphoma. Of the five dogs with follicular lymphoma, two were male (40%) and three were female (60%). The age of affected dogs ranged from 11 to 13 years. Four of the five (80%) dogs were purebred and one (20%) had no defined breed. All dogs presented generalized lymphadenomegaly and splenomegaly, which included cases as multicentric lymphoma. At necropsy, the lymph nodes and the spleen demonstrated a nodular pattern at the cut surface, characterized by tens to hundreds of white nodules of variable size, multifocal or coalescing. On the natural surface of the spleen, often (4/5, 80%), there were myriads of white, multifocal or coalescing dots of varying sizes. In histopathology, the tumors were confirmed as follicular lymphomas. All cases were Grade III, two (40%) included as IIIa and three (60%) as IIIb. In one case (1/5, 20%), follicular lymphoma was considered as a IIIb variant of small centroblasts similar to the neoplastic lymphocytes seen in Burkitt's lymphoma. Lymphomas were validated as having origin in B cells through immunohistochemistry, using anti-CD20 antibody. The cases of follicular lymphomas described behaved aggressively and led the patients to death.(AU)

Transplante de células-tronco hematopoéticas alogênico com regime de condicionamento de intensidade reduzida em linfoma folicular refratário ou recaído: uma alternativa promissora / Allogeneic hematopoietic stem cell transplantation with reduced-intensity conditioning regimen in refractory or relapsed follicular lymphoma: a promising alternative

O linfoma folicular é um tipo de linfoma não Hodgkin de células B indolente. Apenas 30% dos pacientes apresentam doença em fase inicial ao diagnóstico. Os pacientes com estadiamento III-IV estão entre a maioria dos diagnósticos da doença e apresentam altas taxas de recaída ou refratariedade ao tratamento. O linfoma folicular recaído ou refratário permanece um desafio para a prática clínica. O transplante de células-tronco hematopoéticas autólogo vem sendo utilizado há muito tempo nesse perfil de pacientes, com altos índices de complicações como segunda neoplasia e curto período de remissão. O transplante de células-tronco hematopoéticas alogênico com regime de condicionamento mieloablativo apresenta resultados pouco aceitáveis, devido ao aumento da mortalidade relacionada ao tratamento sem benefícios em sobrevida global, da sobrevida livre de doença ou da taxa de recaída que sustentem tal indicação O transplante de células-tronco hematopoéticas alogênico com regime de condicionamento com intensidade reduzida parece ser uma alternativa promissora, inclusive como primeiro transplante. Alguns estudos comparando os resultados dos três tipos de transplantes em pacientes com linfoma folicular recaído ou refratário, com enfoque principal no transplante de células-tronco hematopoéticas alogênico de condicionamento com intensidade reduzida, são descritos neste artigo de revisão.(AU)

Follicular Lymphoma is a type of indolent B-cell non-Hodgkin´s lymphoma. Only 30% of the patients present with an early phase of the disease at diagnosis. Patients with stage III-IV are among the majority of the diagnoses of the disease, and these have high rates of relapse or refractoriness to treatment. Relapsed or refractory follicular lymphoma remains a challenge for clinical practice. Autologous hematopoietic stem cell transplantation has been used for a long time in this profile of patients, with high rates of complications, such as second neoplasia and short remission period. The allogenic hematopoietic stem cell transplantation with myeloablative conditioning regimen presents poorly acceptable results due to increased treatment-related mortality with no overall survival benefits, disease-free survival, or relapse rate to warrant it. Allogeneic hematopoietic stem cell transplantation with reduced-intensity conditioning regimen seems to be a promising alternative, even as the first transplant. Some studies comparing the results of the three types of transplants in patients with relapsed or refractory follicular lymphoma , with a main focus on hematopoietic stem cell transplantation allogenic with reduced-intensity conditioning regimen, will be described in this review article.(AU)

Transformación leucémica en paciente con linfoma folicular e hiperleucocitosis al diagnóstico / Leukemic transformation in a patient with follicular lymphoma and hyperleukocytosis at diagnosis

La fase leucémica como presentación de un linfoma folicular es rara y debe ser considerada factor de mal pronóstico. Por otra parte, la asociación entre linfoma folicular y síndrome mielodisplásico no se ha descrito. Se presenta el caso de una paciente en la que se detectó marcada leucocitosis y a la que se diagnosticó un linfoma folicular. Recibió quimioterapia con R-CHOP y FCR cuando recayó. Meses después, se realizó un aspirado medular en el cual se observaron cambios compatibles con mielodisplasia, únicamente recibió terapia de soporte y finalmente evolucionó a leucemia mieloide aguda. Aunque se conoce que la mielodisplasia puede ser secundaria al uso de quimioterapia, la paciente presentó además trisomía del cromosoma 11, descrita previamente en mielodisplasia y linfoma tipo Burkitt, la cual pudiera estar en relación con la evolución a leucemia mieloide aguda(AU)

Follicular lymphoma rarely presents with a leukemic phase and this should be considered a negative prognostic factor. Also, follicular lymphoma and myelodysplastic syndrome association has not been previously reported. Herein we present a patient who debuted with marked hyperleukocytosis and was diagnosed with follicular lymphoma, receiving CHOP-R and FCR after she relapsed. Several months later, secondary myelodysplastic changes were observed in her bone marrow. She received supportive therapy and finally progressed into acute myeloid leukemia. Although secondary myelodysplasia is known to be produced by chemotherapy, this patient additionally had trisomy 11, previously described in myelodysplasia and Burkitt's lymphoma, which could be linked to progression to acute myeloid leukemia(AU)

Linfoma folicular primario intestinal no polipósico: reporte de un caso y revisión de la literatura / Primary non-polipoid intestinal folicular lymphoma: case report and review of the literature

El linfoma folicular primario intestinal es un desorden raro descrito en la última clasificación de linfomas de la WHO. Es una entidad localizada con excelente pronóstico. En el presente artículo, reportamos una mujer peruana de 64 años de edad diagnosticada con linfoma folicular primario intestinal. Ella tuvo dos años con dolor abdominal y vómitos tardíos. Ella desarrolló una obstrucción intestinal y tuvo una resección completa del tumor. Se describe el caso y se realiza una revisión de la literatura de esta entidad.

The primary intestinal follicular lymphoma is a rare disease described in the last classification of lymphomas from WHO. It is a localized disease with excellent prognosis. We describe in this article ,a 64 year-old Peruvian female with abdominal pain and delayed vomiting for the last two years, has undergone a partial intestinal resection due to bowel obstruction. There was a well-circumscribed annular tumor. A diagnosis of non-polypoid primary intestinal follicular lymphoma was made. We report the case and review the literature in this article.

Linfoma folicular en un adolescente: presentación de un caso / Follicular Lymphoma in a Teenager: A Case Report

El linfoma no Hodgkin de tipo folicular en la edad pediátrica es una patología muy rara, por lo que es difícil encontrar estadísticas epidemiológicas veraces acerca de su presentación. Los linfomas no Hodgkin en general constituyen el 8 % de los casos en niños con patologías neoplásicas, pero su variedad folicular en infantes se encuentra escasamente referenciada por su baja frecuencia. Es relevante el reconocimiento de su abordaje diagnóstico, en el que se destacan la histopatología y el manejo en el servicio de oncohematología pediátrica. Se puede afirmar que el diagnóstico adecuado constituye un reto para el equipo tratante, pues su escasa presentación en esta población específica no aporta herramientas a la sospecha clínica. Este artículo presenta un caso de linfoma folicular en una adolescente de 17 años de edad. Inicialmente se realiza una ecografía hepatobiliar y luego una TAC contrastada. Se encuentra una lesión de centro hipodenso localizada en el hipocondrio derecho; se plantea una neoplasia de contenido necrótico, que se confirma el linfoma folicular con la histopatología e inmunohistoquímica de la muestra de un tumor retroperitoneal obtenida en la intervención quirúrgica.

Follicular type non-Hodgkin's lymphoma is a very rare pathology at a pediatric age. For this reason, it is very difficult to find truthful epidemiological statistics regarding its appearance. Generally speaking, non-Hodgkin's lymphoma constitute 8% of cases in children with neoplastic pathologies. However, its follicular variety in children is scarcely found because of its low frequency. The recognition of its diagnosis is relevant. This diagnosis includes the histopathology and the handling of pediapediatric oncohematology. One can say that making a suitable diagnosis is a challenge for the treating team, given that its scarce presentation in this specific population does not provide tools for the clinic suspicion. This article shows a case of follicular lymphoma in a 17 year old teenager. Initially, a hepatic-biliar sonogram, and then a contrastive CT scan were performed. A located lesion of a dense core is found in the right hypochondriac. A neoplasia of necrotic content is set out, in which a follicular lymphoma is confirmed with the histopathology and immunohistochemistry of a retro peritoneum tumor sample obtained through surgical intervention.

Primary cutaneous centrofollicular lymphoma with a good response to radiotherapy / Linfoma centrofolicular cutaneo primario com boa resposta a radioterapia

Primary cutaneous lymphomas have different clinical behavior and prognosis than systemic lymphomas of similar histological subtype. About 30% of non-Hodgkin lymphomas involve extranodal tissues, the skin being the second most affected body organ after the gastrointestinal tract (approximately 18%). According to the EORTC (European Organization for Research and Treatment of Cancer) classification, the lymphoma centrofollicular is indolent, since the lesions increase in size slowly over the years and spread to extracutaneous sites is uncommon. Based on this, radiotherapy has been considered the treatment of choice by many studies. We present the case of a patient who had cutaneous centrofollicular scalp lymphoma for 1 year and showed good response to radiotherapy.

Os linfomas cutâneos primários têm comportamento clínico e prognóstico diferente dos linfomas sistêmicos de subtipo histológico semelhante. Cerca de 30% dos linfomas não-Hodgkin acometem tecidos extranodais, sendo a pele o segundo órgão mais envolvido após o trato gastrointestinal (aproximadamente 18%). De acordo com a classificação EORTC (European Organization for Research and Treatment of Cancer), o linfoma centrofolicular cutâneo é indolente, uma vez que as lesões aumentam de tamanho lentamente ao longo dos anos e a disseminação para sitios extracutâneos é incomum. Com base nisso, a radioterapia tem sido apontada como o tratamento de primeira escolha por muitos estudos. Apresentamos um caso de paciente com linfoma cutâneo centrofolicular no couro cabeludo há 1 ano e que apresentou boa resposta à radioterapia.

Rol de la terapia de mantenimiento con rituximab en linfoma folicular (LF) / Role of rituximab maintenance therapy in follicular lymphoma

Los LF representan aproximadamente el 25% de los Linfomas no Hodgkin. Debido a su curso clínico indolente y recaídas habituales, son considerados una entidad incurable con los regímenes quimioterápicos convencionales. Aunque el advenimiento del Rituximab modificó la sobrevida global, el tratamiento de mantenimiento con este fármaco retrasa la recaída y consolida la respuesta alcanzada luego de la fase de inducción. Con una dosis semanal de Rituximab de 375 mg/m2 se demostró una acumulación progresiva de su concentración plasmática, la cual continúa siendo detectable luego de 3–6 meses de la última infusión. Existen varios esquemas de mantenimiento que varían con intervalos entre 2, 3 y 6 meses, todos ellos han demostrado efectividad. El más utilizado resulta el trimensual ya que logra mantener niveles séricos de Rituximab mayores a 25 μg/l (valor target en mantenimiento) con una duración de 2 años. Entre los efectos adversos predominan la reagudización de infecciones virales y la disminución de inmunoglobulinas (Ig) (principalmente Ig M), sin un incremento en la mortalidad. Debido a que existe una correlación lineal entre el descenso de Ig y la actividad del Rituximab, la terapéutica debería personalizarse según los niveles de Ig previos a cada ciclo de mantenimiento para optimizar la distancia entre los mismos, ahorrando costos y disminuyendo el riesgo de infección. Además, la detección del rearreglo del BCL2 al inicio y final del mantenimiento definirá la posibilidad de recaída y la necesidad o no de prolongarlo más allá de 2 años, con un probable impacto en la sobrevida global.

Follicular lymphomas account for about 25% of non–Hodgkin lymphomas. Due to its indolent clinical course and common relapses are considered incurable entity with conventional chemotherapy regimens. Although the advent of Rituximab have modified the overall survival, maintenance treatment with this drug, delayed the relapse and consolidates the response reached after the induction’s phase. A weekly dose of 375 mg/m2 of Rituximab showed a progressive accumulation of its plasma concentration, which remains detectable 3–6 months after the last infusion.There are several schemes of maintenance that ranging from 2, 3 and 6 months, all with proved effective. The mostwidely used is the three–monthly since achieved maintain serum Rituximab levels greater than 25 μg/l (target value in maintenance) during two years.Among the adverse effects predominate exacerbation of viral infections and decreased of immunoglobulins (mainlyIgM), without an increase in mortality. Because there is a linear correlation between the decrease in immunoglobulins and the activity of Rituximab, therapy should be individualized to immunoglobulin’s levels before each maintenance cycle in order to optimize the distance between them, saving costs and reducing the risk of infection. Besides, the detection of BCL2 rearrangement at the beginning and end of maintenance would define the possibility of relapse and whether or not extended the maintenance beyond two years, with a likely impact on overall survival.

Linfoma folicular primario duodenal: informe de un caso y revisión de la literatura / Primary follicular lymphoma of the duodenum: case report and literature review

Mujer de 80 años con síntomas dispépticos a quien se le realizó endoscopia de vías digestivas altas que mostró lesiones polipoides confluentes en la segunda porción del duodeno, cuyo estudio histológico indicó un linfoma no Hodgkin B folicular primario, neoplasia duodenal infrecuente de bajo grado, la cual debe diferenciarse de hiperplasia linfoide folicular reactiva y de otros linfomas

Patient was an 80 year old woman with dyspepsia. An upper endoscopy showed confluent polypoid lesions in the second portion of the duodenum. A histological study showed a follicular B-cell lymphoma. This is a rare low grade neoplasm of duodenum which must be differentiated from follicular reactive hyperplasia and other lymphomas

Tejido linfoide y linfomas gástricos / Lymphoid tissue and gastric lymphomas

En este artículo hemos realizado una amplia revisión de los linfomas primarios gástricos, su clasificación y aspectos clínico-patológicos más importantes, haciendo énfasis especial en los linfomas MALT o asociados a las mucosas y su relación con la infección por Helicobacter pylori.

In this review we describe various aspects of the primary gastric lymphomas, the classification, and the most important clinico-pathological aspects, with emphasis in mucosa associated lymphoma (MALT) and the Helicobacter pylori infection.

Risk stratification for indolent lymphomas / Estratificação de risco dos linfomas indolentes

Indolent B-cell lymphomas account for approximately 40 percent of all non-Hodgkin lymphomas (NHLs). Advances in technology have contributed to improvements in the diagnosis and classification of indolent non-Hodgkin lymphomas. Follicular Lymphomas are the most common although the frequency varies significantly throughout the world. The description of the Follicular Lymphoma International Prognostic Index (FLIPI) was an important step in identifying patient subgroups, but its use in the clinical practice has not been established yet. The use of a larger number of paraffin active monoclonal antibodies for immunohistochemistry, molecular cytogenetic studies including standard cytogenetics, multi-color fluorescence in-situ hybridization (FISH), polymerase chain reaction and locus-specific fluorescence insitu hybridization as well as developments in high-resolution techniquesincluding microarray gene expression profiling allow more accurate diagnosis andprecise definition of biomarkers of value in risk stratification. The identification ofdiseasespecific gene lists resulting from expression profiling provides a number ofpotential protein targets that can be validated using immunohistochemistry. Analysesof gene expression profiles or constitutive gene variations may also provide additional insight for prognostication in the near future. A comprehensive understanding of the biology of these distinct lymphoid tumors will allow us to identify novel diseaserelated genes and should facilitate the development of improved diagnosis, outcome prediction, and personalized approaches to treatment.

Os linfomas de células B indolentes representam aproximadamente 40 por cento do total de linfomas não Hodgkin (LNHs). O avanço das tecnologias novas tem contribuído para a melhora no diagnóstico e classificação dos LNH indolentes. O linfoma folicular é o mais comum e sua frequência varia significantemente pelo mundo. Adescrição do Índice Internacional de Prognóstico dos linfomas folicular (FLIPI) representa um passo importante na identificação de subgrupos de pacientes, mas seu uso na prática clínica ainda necessita ser estabelecido. O uso de um número maior de anticorpos monoclonais para imunoistoquímica, estudo citogenético incluindo citogenética convencional ou hibridização in-situ por fluorescência (FISH), bem como o desenvolvimento de técnicas de alta resolução incluindo a expressão por microarray possibilita maior acurácia no diagnóstico e definição precisa dos biomarcadores com valor na estratificação de risco. A identificação de genes específicos para os diversos tipos de linfomas permite o reconhecimento de potenciais proteínas alvo que podem ser validadas usando imunoistoquímica. Análises da expressão do perfil de genes ou variações genéticas constitutivas pode também prover conhecimentos adicionais para o prognóstico em um futuro próximo. Um entendimento da biologia desses distintos tumores linfoides permite-nos identificar novos grupos de genes relacionados à doença e deve facilitar o desenvolvimento diagnóstico, predizendo a evolução e permitindo tratamentos personalizados.