RESUMEN
Rituximab is a chimeric mouse-human antibody that targets the CD20 antigen, which is found in both normal and neoplastic B cells. In recent years, it has been increasingly used to treat cutaneous B-cell lymphoma and is now considered an alternative to classic treatment (radiotherapy and surgery) of 2 types of indolent lymphoma, namely, primary cutaneous follicle center lymphoma and primary cutaneous marginal zone B-cell lymphoma. Rituximab is also administered as an alternative to polychemotherapy in the treatment of primary cutaneous large B-cell lymphoma, leg type. Its use as an alternative drug led to it being administered intralesionally, with beneficial effects. In the present article, we review the literature published on the use of rituximab to treat primary cutaneous B-cell lymphoma.
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Anticuerpos Monoclonales de Origen Murino/uso terapéutico , Antineoplásicos/uso terapéutico , Linfoma de Células B/tratamiento farmacológico , Neoplasias Cutáneas/tratamiento farmacológico , Humanos , RituximabRESUMEN
The objective of the present paper was to review the clinical application of [18F]FDG PET/CT in follicular lymphoma (FL). Once it was clear that, despite it is characterized as indolent, this type of lymphoma usually shows a high [18F]FDG avidity, PET/CT became more important and it's now considered the standard technique in staging, re-staging and response evaluation. Many studies have shown its impact on the management of patients (as it can change the stage in a significant proportion of cases and lead to treatment modifications), its superiority over CT (mainly because it's able to distinguish fibrosis in residual masses from viable tumor) and its prognostic value. The latter was initially associated only to the degree of metabolic response, which has proved to be a strong and independent predictive factor in terms of disease-free survival (DFS) and overall survival (OS). Thus, a negative PET/CT scan could be considered a guarantee in high-tumor-burden follicular lymphoma patients. However, semiquantitative parameters such as metabolic tumor volume or total lesion glycolysis, may also provide useful information and help us to identify patients with poor prognosis, guiding a risk-adjusted management and follow-up.
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Fluorodesoxiglucosa F18 , Linfoma Folicular , Humanos , Linfoma Folicular/diagnóstico por imagen , Tomografía Computarizada por Tomografía de Emisión de Positrones/métodos , Tomografía de Emisión de Positrones , RadiofármacosRESUMEN
Follicular lymphoma, the most common indolent lymphoma, originates from germinal centre B-cells of the lymphoid follicle, and is characterized by t(14;18). Clinical manifestations include the presence of lymphadenopathy, sometimes accompanied by constitutional symptoms or cytopenia. Diagnosis is established through the identification of a B-cell proliferation of nodular pattern in the lymph node biopsy. Upon staging with PET-CT and bone marrow biopsy, a significant proportion of patients do not need immediate treatment. When therapy is indicated, commonly used regimens include anti-CD20 immunotherapy with or without chemotherapy. Although overall survival for most patients is prolonged, relapses are very frequent, and early relapse and transformation to an aggressive lymphoma portend a much worse prognosis. New therapies are under development, which will most likely change outcomes for FL patients in the near future.
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Linfoma Folicular , Linfoma no Hodgkin , Humanos , Linfoma Folicular/diagnóstico , Linfoma Folicular/terapia , Recurrencia Local de Neoplasia , Tomografía Computarizada por Tomografía de Emisión de Positrones , PronósticoRESUMEN
Composite lymphoma with mantle and follicular cell components is a challenging diagnosis. Flow cytometry, immunohistochemistry and molecular genetics are required to distinguish the two components, as often the more aggressive one is predominant and masks the other. A 58-year-old man with history of nodal composite lymphoma presented with right exophthalmos and diplopia. A head CT scan showed an orbital tumor. A biopsy of the tumor revealed a mantle cell lymphoma predominating over a follicular lymphoma. Immunoglobulin heavy chain and light chain rearrangements analysis by PCR proved that both components of the orbital tumor were recurrences of the same nodal composite lymphoma diagnosed two years earlier. The nodal lymphoma was composed of a follicular lymphoma and an in situ mantle cell neoplasia. Consensus view is that dominant lymphoma should be treated when needed but taking into account if the mantle cell lymphoma is an in situ neoplasia and if it expresses CD5 and SOX11.
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Linfoma Compuesto/patología , Linfoma Folicular/patología , Linfoma de Células del Manto/patología , Linfoma/patología , Neoplasias Orbitales/patología , Linfoma Compuesto/química , Linfoma Compuesto/diagnóstico , Humanos , Ganglios Linfáticos/química , Ganglios Linfáticos/patología , Linfoma/química , Linfoma/diagnóstico , Linfoma Folicular/química , Linfoma Folicular/diagnóstico , Linfoma de Células del Manto/química , Linfoma de Células del Manto/diagnóstico , Masculino , Persona de Mediana Edad , Cuello , Neoplasias Orbitales/química , Neoplasias Orbitales/diagnósticoRESUMEN
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.
RESUMEN
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.
RESUMEN
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.
Asunto(s)
Humanos , Masculino , Persona de Mediana Edad , Linfoma no Hodgkin/diagnóstico por imagen , Linfoma Folicular/diagnóstico por imagen , Glándula Tiroides , Costa RicaRESUMEN
CLINICAL CASE: A 49-year-old woman experienced a local relapse of a primary follicular lymphoma (FL) of the conjunctiva. She received 4 weekly intra-lesional injections followed by 6 monthly injections of rituximab (6mg/ml). A clinical response was achieved after first injection. No adverse ocular event or signs of lymphoma relapse were seen after 10 months of follow-up. DISCUSSION: Intralesional administration of rituximab for treating primary FL of the conjunctiva was an effective and safe therapeutic option; therefore it could be an alternative to other conventional treatments, such as radiotherapy or chemotherapy.
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Antineoplásicos Inmunológicos/administración & dosificación , Neoplasias de la Conjuntiva/tratamiento farmacológico , Linfoma Folicular/tratamiento farmacológico , Recurrencia Local de Neoplasia/tratamiento farmacológico , Rituximab/administración & dosificación , Femenino , Humanos , Inyecciones Intralesiones , Persona de Mediana EdadRESUMEN
El objetivo de este trabajo fue revisar el papel de la [18F]FDG PET/TC en el linfoma folicular (LF). Tras confirmarse que a pesar de su carácter indolente este tipo de linfoma habitualmente muestra avidez por el radiotrazador, la [18F]FDG PET/TC fue cobrando una importancia progresivamente mayor hasta ser considerada como la técnica de elección para su estadificación, re-estadificación y valoración de respuesta al tratamiento. Múltiples estudios han demostrado el impacto que supone en el manejo de estos pacientes (puede cambiar el estadio de la enfermedad en una proporción significativa de casos y condicionar modificaciones en el tratamiento), su superioridad respecto a la TC (principalmente por la capacidad para distinguir tejido tumoral viable de tejido fibrótico residual) y su valor pronóstico. Esto último se atribuyó inicialmente de forma exclusiva al grado de respuesta metabólica alcanzado tras el tratamiento, que ha probado ser un factor predictivo fuerte e independiente de supervivencia libre de progresión (SLP) y supervivencia global (SG), de modo que una [18F]FDG PET/TC negativa podría considerarse una garantía para los pacientes con LF con elevada carga tumoral. No obstante, la obtención de parámetros metabólicos semicuantitativos como el volumen metabólico tumoral o la glucólisis total de la lesión podría también aportar información a este respecto y ayudarnos potencialmente a identificar a los pacientes de mal pronóstico antes del inicio del tratamiento, de forma que se pueda adecuar el manejo y seguimiento al riesgo del paciente (AU)
The objective of the present paper was to review the clinical application of [18F]FDG PET/CT in follicular lymphoma (FL). Once it was clear that, despite it's characterized as indolent, this type of lymphoma usually shows a high [18F]FDG avidity, PET/CT became more important and it's now considered the standard technique in staging, re-staging and response evaluation. Many studies have shown its impact on the management of patients (as it can change the stage in a significant proportion of cases and lead to treatment modifications), its superiority over CT (mainly because it's able to distinguish fibrosis in residual masses from viable tumor) and its prognostic value. The latter was initially associated only to the degree of metabolic response, which has proved to be a strong and independent predictive factor in terms of disease-free survival (DFS) and overall survival (OS). Thus, a negative PET/CT scan could be considered a guarantee in high-tumor-burden follicular lymphoma patients. However, semiquantitative parameters such as metabolic tumor volume or total lesion glycolysis, may also provide useful information and help us to identify patients with poor prognosis, guiding a risk-adjusted management and follow-up (AU)
Asunto(s)
Humanos , Linfoma Folicular/diagnóstico por imagen , Fluorodesoxiglucosa F18 , Radiofármacos , Tomografía Computarizada por Tomografía de Emisión de Positrones/métodosRESUMEN
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.
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Humanos , Femenino , Adolescente , Linfoma Folicular/diagnóstico , Linfoma Folicular/patología , Linfoma no Hodgkin/diagnóstico , Linfoma no Hodgkin/patologíaRESUMEN
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.
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Humanos , Femenino , Adulto , Linfoma no Hodgkin , Linfoma Folicular , Tracto Gastrointestinal , Linfoma , Terapéutica , Helicobacter pylori , VIH , Terapia de Inmunosupresión , RituximabRESUMEN
CLINICAL CASE: The case is presented of a 43 year-old male patient with chronic follicular conjunctivitis, negative bacterial serology, and refractory to local treatment. The incisional biopsy performed showed to be consistent with reactive lymphoid hyperplasia. A year later, a new incisional biopsy showed follicular lymphoma, with no systemic involvement, and he was treated with local radiotherapy. DISCUSSION: When a chronic follicular conjunctivitis is refractory to treatment, it is essential to perform an incisional biopsy to establish the histopathological diagnosis that can range from chronic inflammation, reactive lymphoid hyperplasia to lymphoma. Follicular lymphoma is rare among conjunctival lymphomas, and the staging is indispensable for the correct therapeutic approach.
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Neoplasias de la Conjuntiva/diagnóstico , Conjuntivitis/diagnóstico , Linfoma Folicular/diagnóstico , Adulto , Conjuntiva , Diagnóstico Diferencial , Humanos , MasculinoRESUMEN
AIM: To analyze the relationship of clinical variables related to prognosis and tumor burden, with metabolic variables obtained in the staging (18)F-FDG PET/CT, and their value in the prognosis in follicular lymphoma (FL). METHODS: 82 patients with FL, a (18)F-FDG PET/CT at diagnosis and a follow-up for a minimum of 12 months, were retrospectively enrolled in the present study. Clinical variables (Tumor grade, Follicular Lymphoma International Prognostic Index (FLIPI) and Tumor burden) were evaluated. Metabolic variables such as SUVmax in the highest hypermetabolic lesion, extralymphatic locations, number of involved lymph node locations, bone marrow (BM) involvement, PET stage and diameter of the biggest hypermetabolic lesion, were analyzed in order to establish a PET score and classify the studies in low, intermediate and high metabolic risk. Clinical and metabolic variables (included metabolic risk) were compared. The relation among all variables and disease-free survival (DFS) was studied. RESULTS: The 28% of patients had a high-grade tumor. The 30.5% had FLIPI risk low, 29.3% intermediate y 40.2% high. The 42.7% presented a high tumor burden. The PET/CT was positive in 94% of patients. The tumor grade did not show significant relation with metabolic variable. FLIPI risk and tumor burden showed statistical relations with the SUV max and the PET score (p<0.008 and p=0.003 respectively). With respect to DFS, significant differences were detected for the PET stage and FLIPI risk (p=0.015 and p=0.047 respectively). FLIPI risk was the only significant predictor in Cox regression analysis, with a Hazard Ratio of 5.13 between high risk and low risk. CONCLUSION: The present research highlights the significant relation between metabolic variables obtained with FDG PET/CT and clinical variables although their goal as an independent factor of prognosis was not demonstrated in the present work.
Asunto(s)
Fluorodesoxiglucosa F18 , Linfoma Folicular/diagnóstico por imagen , Linfoma Folicular/metabolismo , Tomografía Computarizada por Tomografía de Emisión de Positrones , Radiofármacos , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Supervivencia sin Enfermedad , Femenino , Humanos , Estudios Longitudinales , Masculino , Persona de Mediana Edad , Pronóstico , Estudios Retrospectivos , Carga Tumoral , Adulto JovenRESUMEN
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.
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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.
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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.
RESUMEN
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.
RESUMEN
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)
Asunto(s)
Humanos , Masculino , Femenino , Antibióticos Antineoplásicos/administración & dosificación , Trasplante de Células Madre Hematopoyéticas/métodos , Linfoma Folicular/patología , Linfoma Folicular/terapia , Trasplante Autólogo/métodosRESUMEN
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)
Asunto(s)
Humanos , Femenino , Adulto , Leucemia/mortalidad , Linfoma Folicular/complicaciones , Leucocitosis/complicaciones , Linfoma FolicularRESUMEN
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)