Survival Analysis and Prognostic Factors in a Case Series of 148 Cutaneous T-Cell Lymphomas. / Análisis de supervivencia y factores pronósticos en una serie de 148 casos de linfomas cutáneos de células T.

BACKGROUND AND OBJECTIVE: Cutaneous T-cell lymphomas (CTCL) such as mycosis fungoides (MF) and Sézary syndrome (SS) are rare lymphomas with varying prognoses. The aim of the study was to describe the survival of a cohort of patients with MF/SS and evaluate the prognostic factors impacting disease survival. MATERIALS AND METHODS: All cases of MF/SS diagnosed from 2008 through 2022 were retrospectively analyzed. The demographic variables, histological parameters, and analytical data were analyzed too. Progression-free survival (PFS) and disease-specific survival (DSS) were calculated. RESULTS: A total of 148 cases were included. A total of 121 (82%) and 27 cases were diagnosed with MF, and SS, respectively. A total of 37 patients (25%) experienced progression at some point disease progression. The median PFS and median DSS were 127 and 135 months, respectively. Age >60 years, diagnosis of SS, the presence of large cell transformation (LCT) at diagnosis, folliculotropism in early stages, high Ki-67 expression, the presence of the clonal T-cell receptor (TCR) in blood, elevated LDH and B2M levels, and advanced stages (IIB, IVA, T3, T4, N3/Nx) were associated with worse prognosis across the entire cohort. CONCLUSIONS: Stage IVA and the presence of LCT at diagnosis stood out as independent factors of unfavorable prognosis. LCT was the variable that most significantly impacted the patients' survival and was closely associated with tumor skin involvement and stage IIB.

Reporte preliminar de las características sociodemográficas y clínicas de los pacientes con hemopatías malignas: experiencia de un hospital universitario

Introducción: debido a su amplia diversidad clínica y biológica, es de gran importancia para el médico conocer cómo se presentan los pacientes diagnosticados con una hemopatía maligna. Objetivo: describir las características sociodemográficas y clínicas de los pacientes con diagnóstico de una hemopatía maligna que consultaron en un Hospital Universitario de Paraguay. Metodología: estudio observacional, retrospectivo, descriptivo, de corte transversal, con muestreo no probabilístico, de casos consecutivos. Se incluyó a pacientes adultos, de ambos sexos, registrados en el archivo de Hematología del Hospital de Clínicas de la Facultad de Ciencias Médicas de la Universidad Nacional de Asunción (Paraguay), con diagnóstico confirmado por anatomía patológica de una neoplasia hematológica, según la clasificación de la Organización Mundial de la Salud, durante el periodo comprendido entre enero 2019 y agosto de 2021. Se estudiaron variables sociodemográficas y clínicas. Resultados: se estudió a 129 pacientes. El 58 % correspondía al sexo masculino. La edad promedio de los pacientes fue de 50,3±17,38. La mayoría de los pacientes procedía del Departamento Central de Paraguay (56,58 %). La mayoría de los pacientes (n=37) fue diagnosticado con un linfoma de tipo no Hodgkin o con una neoplasia con origen en células plasmáticas (n=32). El tiempo promedio de aparición de los síntomas hasta la consulta fue de 3,5 meses. El síntoma más reportado fue la astenia (35 %). La palidez fue el hallazgo positivo más frecuente al examen físico. Las comorbilidades más frecuentes fueron la hipertensión arterial (39,44 %) y la diabetes mellitus (15,59 %). Discusión: el paciente típico con una hemopatía que consulta en el Hospital de Clínicas es hombre, mayor de 50 años, proveniente del Departamento Central, consulta por astenia, presenta palidez al examen físico y ha tardado más de 3 meses en consultar. Se le ha diagnosticado un linfoma no Hodgkin y tiene comorbilidades cardiovasculares o metabólicas.

Introduction: due to their wide clinical and biological diversity, it is of great importance for the physician to know how patients diagnosed with a hematological malignancy present. Objective: to describe the sociodemographic and clinical characteristics of patients diagnosed with a hematological malignancy who consulted at a University Hospital in Paraguay. Methods: observational, retrospective, descriptive, cross-sectional, retrospective, descriptive, cross-sectional study, with non-probabilistic sampling of consecutive cases. We included adult patients, of both sexes, registered in the Hematology file of the Hospital de Clínicas of the Faculty of Medical Sciences of the National University of Asunción (Paraguay), with diagnosis confirmed by pathological anatomy of a hematological neoplasm, according to the classification of the World Health Organization, during the period from January 2019 to August 2021. Sociodemographic and clinical variables were studied. Results: 129 patients were studied. Fifty-eight percent were male. The mean age of the patients was 50.3±17.38. Most of the patients were from the Central Department of Paraguay (56.58 %). Most of the patients (n=37) were diagnosed with a non-Hodgkin's lymphoma or a neoplasm with plasma cell origin (n=32). The average time from symptom onset to consultation was 3.5 months. The most reported symptom was asthenia (35%). Pallor was the most frequent positive finding on physical examination. The most frequent comorbidities were arterial hypertension (39.44%) and diabetes mellitus (15.59%). Discussion: the typical patient with a hemopathy who consults at the Hospital de Clínicas is male, over 50 years of age, from the Central Department, consults for asthenia, presents pallor on physical examination and has taken more than 3 months to consult. He has been diagnosed with non-Hodgkin's lymphoma and has cardiovascular or metabolic comorbidities.

Registro de linfomas cutáneos primarios (RELCP) de la AEDV: datos tras 5 años de funcionamiento / Primary Cutaneous Lymphoma Registry of the Spanish Academy of Dermatology and Venereology (AEDV): Data for the First 5 Years

Antecedentes y objetivos Los linfomas cutáneos primarios (LCP) son un conjunto de entidades poco frecuentes. En febrero del 2018 se describieron los resultados del primer año de funcionamiento del Registro de linfomas cutáneos primarios de la AEDV. En el presente trabajo actualizamos los resultados tras 5 años de funcionamiento. Pacientes y métodos Registro de enfermedad de pacientes con LCP. Se recogieron datos prospectivamente de los pacientes, incluyendo diagnóstico, tratamientos, pruebas realizadas y estado actual del paciente. Se realizó un análisis descriptivo. Resultados En diciembre del 2021 se había incluido a un total de 2020 pacientes en el Registro, pertenecientes a 33 hospitales españoles. El 59% fueron hombres, la edad media fue de 62,2 años. Se agruparon en 4grandes grupos diagnósticos: micosis fungoide/síndrome de Sézary (1.112, 55%), LCP de células B (547, 27,1%), trastornos linfoproliferativos de células T CD30+(222, 11%) y otros linfomas T (116, 5,8%). La mayoría presentó estadio T1, encontrándose actualmente casi el 75% en remisión completa (43,5%) o enfermedad estable (EE; 27%). Los tratamientos más usados fueron corticoides tópicos (1.369, 67,8%), fototerapia (890, 44,1%), cirugía (412, 20,4%) y radioterapia (384, 19%). Conclusión Las características del paciente con LCP en España no difieren de otras series. El mayor tamaño del registro permite precisar mejor los datos con respecto a los resultados del primer año. Este registro facilita al grupo de linfomas de la AEDV realizar investigación clínica, surgiendo ya trabajos publicados de dicho registro (AU)

Background and objective Primary cutaneous lymphomas (PCL) are uncommon. Observations based on the first year of data from the Spanish Registry of Primary Cutaneous Lymphomas (RELCP, in its Spanish abbreviation) of the Spanish Academy of Dermatology and Venereology (AEDV) were published in February 2018. This report covers RELCP data for the first 5 years. Patients and methods RELCP data were collected prospectively and included diagnosis, treatments, tests, and the current status of patients. We compiled descriptive statistics of the data registered during the first 5 years. Results Information on 2020 patients treated at 33 Spanish hospitals had been included in the RELCP by December 2021. Fifty-nine percent of the patients were men; the mean age was 62.2 years. The lymphomas were grouped into 4 large diagnostic categories: mycosis fungoides/Sézary syndrome, 1112 patients (55%); primary B-cell cutaneous lymphoma, 547 patients (27.1%); primary CD30+lymphoproliferative disorders, 222 patients (11%), and other T-cell lymphomas, 116 patients (5.8%). Nearly 75% of the tumors were registered in stage I. After treatment, 43.5% achieved complete remission and 27% were stable at the time of writing. Treatments prescribed were topical corticosteroids (1369 [67.8%]), phototherapy (890 patients [44.1%]), surgery (412 patients [20.4%]), and radiotherapy (384 patients [19%]). Conclusion The characteristics of cutaneous lymphomas in Spain are similar to those reported for other series. The large size of the RELCP registry at 5 years has allowed us to give more precise descriptive statistics than in the first year. This registry facilitates the clinical research of the AEDV's lymphoma interest group, which has already published articles based on the RELCP data (AU)

[Translated article] Primary Cutaneous Lymphoma Registry of the Spanish Academy of Dermatology and Venereology (AEDV): Data for the First 5 Years / Registro de linfomas cutáneos primarios (RELCP) de la AEDV: datos tras 5 años de funcionamiento

Background and objective Primary cutaneous lymphomas (PCL) are uncommon. Observations based on the first year of data from the Spanish Registry of Primary Cutaneous Lymphomas (RELCP, in its Spanish abbreviation) of the Spanish Academy of Dermatology and Venereology (AEDV) were published in February 2018. This report covers RELCP data for the first 5 years. Patients and methods RELCP data were collected prospectively and included diagnosis, treatments, tests, and the current status of patients. We compiled descriptive statistics of the data registered during the first 5 years. Results Information on 2020 patients treated at 33 Spanish hospitals had been included in the RELCP by December 2021. Fifty-nine percent of the patients were men; the mean age was 62.2 years. The lymphomas were grouped into 4 large diagnostic categories: mycosis fungoides/Sézary syndrome, 1112 patients (55%); primary B-cell cutaneous lymphoma, 547 patients (27.1%); primary CD30+lymphoproliferative disorders, 222 patients (11%), and other T-cell lymphomas, 116 patients (5.8%). Nearly 75% of the tumors were registered in stage I. After treatment, 43.5% achieved complete remission and 27% were stable at the time of writing. Treatments prescribed were topical corticosteroids (1369 [67.8%]), phototherapy (890 patients [44.1%]), surgery (412 patients [20.4%]), and radiotherapy (384 patients [19%]). Conclusion The characteristics of cutaneous lymphomas in Spain are similar to those reported for other series. The large size of the RELCP registry at 5 years has allowed us to give more precise descriptive statistics than in the first year. This registry facilitates the clinical research of the AEDV's lymphoma interest group, which has already published articles based on the RELCP data (AU)

Antecedentes y objetivos Los linfomas cutáneos primarios (LCP) son un conjunto de entidades poco frecuentes. En febrero del 2018 se describieron los resultados del primer año de funcionamiento del Registro de linfomas cutáneos primarios de la AEDV. En el presente trabajo actualizamos los resultados tras 5 años de funcionamiento. Pacientes y métodos Registro de enfermedad de pacientes con LCP. Se recogieron datos prospectivamente de los pacientes, incluyendo diagnóstico, tratamientos, pruebas realizadas y estado actual del paciente. Se realizó un análisis descriptivo. Resultados En diciembre del 2021 se había incluido a un total de 2020 pacientes en el Registro, pertenecientes a 33 hospitales españoles. El 59% fueron hombres, la edad media fue de 62,2 años. Se agruparon en 4grandes grupos diagnósticos: micosis fungoide/síndrome de Sézary (1.112, 55%), LCP de células B (547, 27,1%), trastornos linfoproliferativos de células T CD30+(222, 11%) y otros linfomas T (116, 5,8%). La mayoría presentó estadio T1, encontrándose actualmente casi el 75% en remisión completa (43,5%) o enfermedad estable (EE; 27%). Los tratamientos más usados fueron corticoides tópicos (1.369, 67,8%), fototerapia (890, 44,1%), cirugía (412, 20,4%) y radioterapia (384, 19%). Conclusión Las características del paciente con LCP en España no difieren de otras series. El mayor tamaño del registro permite precisar mejor los datos con respecto a los resultados del primer año. Este registro facilita al grupo de linfomas de la AEDV realizar investigación clínica, surgiendo ya trabajos publicados de dicho registro (AU)

Análisis estadístico implicativo para identificar factores pronósticos de mortalidad por linfomas en niños y adolescentes

Fundamento: el análisis estadístico implicativo surgió en los años 80 para resolver problemas de la didáctica de las matemáticas. Recientemente se fundamentó su empleo en las Ciencias Médicas para identificar factores de riesgo y pronósticos. Objetivo: evaluar la utilidad del análisis estadístico implicativo en la identificación de los factores pronósticos que más inciden en la mortalidad por linfomas en niños y adolescentes. Método: se realizó un estudio de casos y controles en niños y adolescentes con diagnóstico de linfoma Hodgkin y no Hodgkin atendidos en el Hospital Docente Pediátrico Sur Dr. Antonio María Béguez César de Santiago de Cuba en el período de enero 2008 a enero 2021. Se analizó como variable dependiente el estado del paciente fallecido o vivo al momento del estudio y como covariables se tomaron: el estadio de mal pronóstico, la presencia de síntomas B, el subtipo histológico, la presencia de tres o más sitios extraganglionares, la metástasis, edad y presencia de masa tumoral. Se aplicaron dos técnicas estadísticas, la regresión logística binaria y el análisis estadístico implicativo. Resultados en los casos fue más frecuente el linfoma no Hodgkin mientras que en los controles predominó el Hodgkin. Ambas técnicas reconocieron el subtipo histológico y la afectación extraganglionar como factores pronósticos desfavorables. El análisis estadístico implicativo reconoció además el estadio y la presencia de metástasis. Conclusión: el análisis estadístico implicativo es una técnica que complementa la regresión logística binaria en la identificación de factores pronósticos, lo que permite mejor comprensión de la causalidad.

Background: the implicative statistical analysis arose in the 80s to solve problems in the didactics of mathematics. Its use in the Medical Sciences to identify risk factors and prognoses was recently founded. Objective: to evaluate the usefulness of the implicative statistical analysis in the identification of the prognostic factors that most affect mortality from lymphomas in children and adolescents. Method: a case-control study was carried out in children and adolescents diagnosed with Hodgkin and non-Hodgkin lymphoma treated at the Dr. Antonio María Béguez César Sur Pediatric Teaching Hospital in Santiago de Cuba from January 2008 to January 2021. The state of the deceased or alive patient at the time of the study was analyzed as the dependent variable and the following were taken as covariates: poor prognosis stage, presence of B symptoms, histological subtype, presence of three or more extranodal sites, metastasis, age and presence of tumor mass. Two statistical techniques were applied: binary logistic regression and implicative statistical analysis. Results: non-Hodgkin's lymphoma was more frequent in the cases, while Hodgkin's lymphoma predominated in the controls. Both techniques recognized the histological subtype and extranodal involvement as unfavorable prognostic factors. The implicative statistical analysis also recognized the stage and the presence of metastases. Conclusion: the implicative statistical analysis is a technique that complements the binary logistic regression in the identification of prognostic factors, which allows a better understanding of causality.

Primary Cutaneous Lymphoma Registry of the Spanish Academy of Dermatology and Venereology (AEDV): Data for the First 5 Years. / Registro de linfomas cutáneos primarios (RELCP) de la AEDV: datos tras 5 años de funcionamiento.

BACKGROUND AND OBJECTIVE: Primary cutaneous lymphomas (PCL) are uncommon. Observations based on the first year of data from the Spanish Registry of Primary Cutaneous Lymphomas (RELCP, in its Spanish abbreviation) of the Spanish Academy of Dermatology and Venereology (AEDV) were published in February 2018. This report covers RELCP data for the first 5 years. PATIENTS AND METHODS: RELCP data were collected prospectively and included diagnosis, treatments, tests, and the current status of patients. We compiled descriptive statistics of the data registered during the first 5 years. RESULTS: Information on 2020 patients treated at 33 Spanish hospitals had been included in the RELCP by December 2021. Fifty-nine percent of the patients were men; the mean age was 62.2 years. The lymphomas were grouped into 4 large diagnostic categories: mycosis fungoides/Sézary syndrome, 1112 patients (55%); primary B-cell cutaneous lymphoma, 547 patients (27.1%); primary CD30+lymphoproliferative disorders, 222 patients (11%), and other T-cell lymphomas, 116 patients (5.8%). Nearly 75% of the tumors were registered in stage I. After treatment, 43.5% achieved complete remission and 27% were stable at the time of writing. Treatments prescribed were topical corticosteroids (1369 [67.8%]), phototherapy (890 patients [44.1%]), surgery (412 patients [20.4%]), and radiotherapy (384 patients [19%]). CONCLUSION: The characteristics of cutaneous lymphomas in Spain are similar to those reported for other series. The large size of the RELCP registry at 5 years has allowed us to give more precise descriptive statistics than in the first year. This registry facilitates the clinical research of the AEDV's lymphoma interest group, which has already published articles based on the RELCP data.

Cutaneous Lymphomas -Part II: Other Cutaneous Lymphomas. / Linfomas cutáneos. Parte II: otros linfomas cutáneos.

Primary cutaneous T-cell lymphomas other than mycosis fungoides, Sézary syndrome, and lymphoproliferative CD30+ disorders are few, accounting for less than 5% of all cutaneous lymphomas. A cytotoxic phenotype is characteristic of these tumors, and their clinical behavior is usually aggressive. Patients often present with extracutaneous symptoms or develop them shortly after diagnosis. Management is usually multidisciplinary, and intensive systemic therapy and bone marrow transplantation should be considered. Cutaneous B-cell lymphomas account for approximately 30% of primary cutaneous lymphomas. They make up a heterogeneous group of tumors that have different clinical and pathological features. Clinical course also varies. Presenting as papules, nodules, or tumors of variable reddish-violaceous coloring, the lesions may be solitary or multiple and occasionally form clusters. There may also be generalized lesions, present at multiple sites on the trunk, head, or extremities. Three well-defined groups of primary cutaneous lymphoma have been reported: follicle center lymphoma; marginal zone lymphoma, which follows an indolent course; and a diffuse large B-cell lymphoma, leg type, which follows an aggressive course.

Cutaneous Lymphomas - Part I: Mycosis Fungoides, Sézary Syndrome, and CD30+ Cutaneous Lymphoproliferative Disorders. / Linfomas cutáneos. Parte I: micosis fungoide, síndrome de Sézary y proliferaciones linfoides cutáneas CD30 positivas.

CD30+ primary cutaneous lymphomas comprise a large group of malignant lymphoproliferative disorders that present in the skin without extracutaneous involvement at the time of diagnosis. The incidence of these lymphomas is low, at 7 to 10 cases per 100 000 population. Two types, derived from T cells (70%-85%) or B cells (15%-30%), have been identified. Hematologists and oncologists have increasingly recognized the idiosyncrasy of primary cutaneous lymphomas, as reflected in the updated classification of the World Health Organization. However, there remain nuances or small differences to consider when managing these conditions, obliging dermatologists to continue to strive to fully reconcile the various clinical pictures in future reviews of the classification of lymphoid neoplasms. A diagnosis of a primary cutaneous lymphoma is based on clinical, histopathologic, immunophenotypic, and genotypic criteria, particularly evidence of T- or B-cell lymphoid monoclonality in lesions. Also relevant are complementary tests to rule out extracutaneous involvement.

Linfoma cutáneo primario de células B. Presentación de un caso / Primary skin lymphoma of cells B. Presentation of a case / Linfoma de células da pele primária B. Apresentação de um caso

RESUMEN Introducción: el Linfoma cutáneo primario del centro folicular es indolente; la supervivencia es de 95% a cinco años; es el más común de los LCCB (35%). Tiene predilección por varones adultos mayores. Se manifiesta con pápulas, placas y tumores solitarios o agrupados principalmente en la piel cabelluda, la frente y el tronco. El objetivo de este trabajo es la presentación del caso por lo inusual de aparición en esta localización y ser el 1ero en nuestro servicio y provincia. Caso clínico: paciente JVL de 58 años de edad, que se interconsulta con los médicos del servicio de Dermatología por presentar lesiones en cuero cabelludo de 3 meses de evolución. Examen físico: Presenta lesiones localizadas en el cuero cabelludo, tumorales en número de 4 redondeadas eritemato violáceas de centro ulcerado con secreción serohemáticas y telangiectasias en su superficie las cuales eran dolorosas. Adenopatías múltiples, pequeñas, movibles en región cervical bilaterales. Se le realizó estudio histopatológico de los tumores para confirmar el diagnóstico clínico planteado. Discusión: los linfomas son tumores malignos del sistema linforreticular, representan un 25% del total de los LCP. Esta neoplasia aparece fundamentalmente entre los 40 y 60 años y es 2,2 veces más frecuente en el sexo masculino que en el femenino, en nuestro caso la paciente tenía 58 años de edad. Si bien es cierto que esta patología es infrecuente, en nuestro caso aparece en mujer con lesiones en cuero cabelludo en una de las localizaciones menos reportada, pero con las lesiones clásicas de los linfomas cutáneos primarios de células B, la tendencia a las ulceraciones se pueden encontrar en la forma centro folicular y en los linfomas difuso de células B como en nuestro caso. Conclusiones: realizamos esta presentación de linfoma cutáneo primario de células B enfermedad infrecuente que se ve mayormente en sexo masculino sin embargo en su variedad difuso se observa más frecuente en mujer en cuero cabelludo de forma ulcerada como apareció en nuestro caso y por ser el primero diagnosticado nuestra provincia.

ABSTRACT Introduction: the primary cutaneous lymphoma of the follicular center is indolent; 95% survival over five years; is the most common LCCB (35%). They have a predilection for larger adult males. It manifests with papules, plaques and solitary tumors or grouped mainly in the skinned hair, in front of the trunk. The purpose of this work is the presentation of the case due to the unusual appearance in this location and to be the first in our service and province. Clinical case: JVL patient of 58 years of age, who is consulted with doctors at the Dermatology service for presenting injuries in the hair of 3 months of evolution. Physical examination: There are lesions located in the hair, tumors in the number of 4 rounded violet erythema of the ulcerated center with serohemic secretion and telangiectasias on their surface that are painful. Multiple, small adenopathies, movable in bilateral cervical region. The histopathological study of the tumors was carried out to confirm the planted clinical diagnosis. Discussion: lymphomas are malignant tumors of the lymphorecticular system, representing 25% of the total PCL. This neoplasm appears fundamentally between 40 and 60 years and 2.2 times more often in men than in women, in case the patient had 58 years of age. If I believe that this pathology is unheard of, in one case it appears in many with lesions in the hair in one of the least reported locations, but with the classic lesions of primary cutaneous B-cell lymphomas, the tendency to ulcers is reduced. find in the form of follicular center and diffuse B-cell lymphomas as in this case. Conclusions: we present this presentation of primary cutaneous B-cell lymphoma that is infrequently ill, seen mostly in men, but in a diffuse variety, it is observed more frequently in the hair in an ulcerated form as it appears in our case and because it is the first diagnosed in our province.

RESUMO Introdução: o linfoma cutâneo primário do centro folicular é indolente; a sobrevivência é de 95% em 5 anos; é o mais comum dos LCCBs (35%). Ele tem predileção por homens adultos mais velhos. Manifesta-se na forma de pápulas, placas e tumores, solitários ou agrupados, principalmente no couro cabeludo, testa e tronco. O objetivo deste trabalho é apresentar o caso devido à sua peculiaridade neste local e ser o primeiro em nosso serviço e província. Caso clínico: paciente JVL, 58 anos, que é consultado pelos médicos do serviço de Dermatologia por apresentar lesões no couro cabeludo com 3 meses de evolução. Exame físico: Apresenta lesões localizadas no couro cabeludo, tumores em número de 4 eritematosos violáceos arredondados com centro ulcerado com secreções cerimônicas e telangiectasias dolorosas na superfície. Linfadenopatia múltipla, pequena e móvel na região cervical, bilateral. O estudo histopatológico dos tumores foi realizado para confirmação do diagnóstico clínico. Discussão: linfomas são tumores malignos do sistema reticular linfático, correspondendo a 25% de todos os LCP. Essa neoplasia surge principalmente entre 40 e 60 anos e é 2,2 vezes mais frequente em homens do que em mulheres, em nosso caso o paciente tinha 58 anos. Embora seja verdade que essa patologia não seja frequente, em nosso caso ela aparece em mulheres com lesões no couro cabeludo em uma das localizações menos relatadas, mas com as lesões clássicas dos linfomas cutâneos primários de células B, a tendência à ulceração é pode ser encontrado no centro folicular e nos linfomas difusos de células B, como em nosso caso. Conclusões: apresentamos esta apresentação do linfoma cutâneo primário de células B, uma doença rara observada principalmente no sexo masculino, mas em sua variedade difusa é observada mais frequentemente em mulheres no couro cabeludo de forma ulcerada como apareceu em nosso caso e por seja o primeiro diagnóstico em nossa província.

Uncommon anatomical sites and anatomopathological presentations of lymphoma in dogs / Sítios anatômicos e apresentações anatomopatológicas incomuns do linfoma em cães

Lymphomas are the tumors most frequently associated with the death or euthanasia of dogs in most parts of the world. In dogs, they almost always occur as disseminated (multicentric lymphoma), gastrointestinal (alimentary lymphoma), or nodal mediastinal (mediastinal lymphoma) diseases. However, other uncommon presentations can occasionally occur. This study aimed to establish the prevalence of these unusual types of lymphoma and demonstrate to veterinary pathologists how they present pathologically. From a total of 100 cases of lymphoma in dogs diagnosed between 1965 and 2017, 16 cases (16/100) were considered by us as non-traditional presentations of the disease: follicular lymphoma, (5/100), peripheral T-cell lymphoma, NOS (2/100), angiocentric lymphoma (2/100), intravascular large T-cell lymphoma (2/100), lymphomatoid granulomatosis (1/100), anaplastic large-cell lymphoma (1/100), hepatosplenic T-cell lymphoma (1/100), and chronic small B-cell lymphocytic lymphoma, intermediate type (1/100). We hope that the results presented here can help veterinary pathologists to recognize such cases of "atypical lymphoma" in their diagnostic routines.(AU)

Linfomas são os tumores mais associados a morte ou eutanásia de cães na maior parte do mundo. Nessa espécie animal ocorrem quase sempre como uma doença disseminada (linfoma multicêntrico), gastrintestinal (linfoma alimentar) ou nodal mediastinal (linfoma mediastínico), entretanto, ocasionalmente, outras apresentações bem menos comuns podem ser encontradas. O objetivo deste artigo é estabelecer a prevalência desses pouco usuais tipos de linfoma e demonstrar aos patologistas veterinários como eles se apresentam anatomopatologicamente. De um total de 100 casos de linfoma em cães diagnosticados entre os anos de 1965 e 2017, 16 casos (16/100) foram considerados como apresentações não tradicionais da doença: linfoma folicular (5/100), linfoma de células T periférico inespecífico (2/100), linfoma angiocêntrico (2/100), linfoma intravascular de grandes células T (2/100), granulomatose linfomatoide (2/100), linfoma de grandes células anaplásicas (1/100), linfoma hepatoesplênico de células T (1/100) e linfoma linfocítico crônico de pequenas células B - tipo intermediário (1/100). Esperamos que os resultados aqui demonstrados auxiliem patologistas veterinários a reconhecerem tais casos de "linfomas atípicos" em suas rotinas diagnósticas.(AU)

Linfoma Cutáneo de Células B Centrofolicular / Centrofollicular B-cell cutaneous lymphoma

Resumen Los Linfomas cutáneos son proliferaciones clonales de Linfocitos T o B neoplásicos. Los linfomas cutáneos B son un grupo heterogéneo de linfomas que se presentan en la piel sin evidencia de compromiso extra cutáneo al momento del diagnóstico y corresponden entre el 20% al 25 % de los linfomas cutáneos primarios.Se presenta un paciente masculino de 71 años, con un linfoma cutáneo de células B centrofolicular localizado en dorso.

Abstract Cutaneous lymphomas are clonal proliferations of neoplastic T or B lymphocytes. Cutaneous B lymphomas are a heterogeneous group of lymphomas presented in the skin without evidence of extra cutaneous harm at the moment of diagnosis and correspond between the 20% and the 25% of primary cutaneous lymphomas. In the current research, a 71 year old masculine patient case is presented, with a cutaneous lymphoma of centrofollicular B cells located at the back.

Tomografía por emisión de positrones asociada con tomografía computarizada en la estadificación de los linfomas / Positron emission tomography combined with computed tomography for the staging of lymphomas

Introducción: Los estudios de imágenes son esenciales para la estadificación de los linfomas. La utilización de la imagen funcional que proporciona la tomografía por emisión de positrones con 18F-2-deoxi-2-fluoro-D-glucosa asociada a la tomografía computarizada ha cambiado fundamentalmente el concepto de estadificación y reestadificación de los linfomas. Constituye una prueba diagnóstica que ha ganado aceptación universal, sobre todo después de la publicación y adopción de las guías de Lugano. Objetivo: Analizar la importancia que tienen las imágenes que proporciona la tomografía por emisión de positrones con 18F-2-deoxi-2-fluoro-D-glucosa asociada a la tomografía computarizada en la estadificación actual de los linfomas. Métodos: Se realizó una revisión bibliográfica, en español y en inglés, de la última década. Se utilizaron los motores de búsqueda de Pubmed, Google y SciELO. Se recolectó y organizó la información siguiendo cronológicamente la aparición de las innovaciones para facilitar la estadificación de los linfomas. Análisis y síntesis de la información: Se hace un recorrido desde la introducción de la tomografía computarizada, la tomografía por emisión de positrones y la asociación de estas, hasta su aplicación en el estudio de los linfomas. Se describe la evolución de los sistemas de clasificación para los linfomas y la utilidad del empleo de la tomografía por emisión de positrones con 18F-2-deoxi-2-fluoro-D-glucosa asociada a la tomografía computarizada en la estadificación de los linfomas. Conclusiones: Es de gran importancia que, en el momento actual, el manejo óptimo de un paciente con linfoma ávido de 18F-2-deoxi-2-fluoro-D-glucosa incluya la estadificación inicial con tomografía por emisión de positrones asociada con tomografía computarizada. Esto permitirá hacer más precisa la etapificación inicial del paciente, optimizar su tratamiento y evaluación de la terapia implementada; así como un mejor pronóstico y evitar estudios invasivos(AU)

Introduction: Imaging studies are essential for staging of lymphomas. The usage of functional imaging provided by positron emission tomography with 18F-2-deoxy-2-fluoro-D-glucose combined with computed tomography has fundamentally changed the concept of staging and re-staging of lymphomas. It constitutes a diagnostic test that has gained universal acceptance, especially after the publication and adoption of the Lugano guidelines. Objective: To analyze the importance of the images provided by positron emission tomography with 18F-2-deoxy-2-fluoro-D-glucose combined with computed tomography in current staging of lymphomas. Methods: A bibliographic review was carried out, in Spanish and in English, within the last decade. We used the search engines of Pubmed, Google, and SciELO. The information was collected and organized by chronologically following the origin of the innovations that facilitate the staging of lymphomas. Information analysis and synthesis: An analysis is carried out from the introduction of computed tomography, positron emission tomography, and the combination of both, to their application in the study of lymphomas. We described the evolution of lymphoma classification systems and the usefulness of positron emission tomography with 18F-2-deoxy-2-fluoro-D-glucose combined with computed tomography for the staging of lymphomas. Conclusions: At the present time, it is of great importance for a patient with lymphoma needing 18F-2-deoxy-2-fluoro-D-glucose to receive optimal management of his or her condition, including initial staging with positron emission tomography combined with computed tomography. This will allow to make the initial staging of the patient more precise, to optimize his or her treatment and evaluation of the implemented therapy, as well as to obtain a better prognosis, avoiding invasive studies(AU)

Supervivencia de pacientes con linfoma no-Hodgkin y síntomas neurológicos según inmunofenotipo del líquido cefalorraquídeo / Survival in non-Hodgkin´s lymphoma patients with neurological symptoms according to the cerebrospinal fluid immunophenotype

Introducción: Los linfomas no-Hodgkin pueden infiltrar el sistema nervioso central y producir síntomas neurológicos, lo cual incrementa la mortalidad. El diagnóstico de esta infiltración se puede realizar mediante el estudio del líquido cefalorraquídeo por la técnica de citometría de flujo, con una mayor sensibilidad que la citología convencional. Objetivo: Estimar la supervivencia global de pacientes con Linfoma no-Hodgkin y síntomas neurológicos según el inmunofenotipo celular del líquido cefalorraquídeo. Métodos: Se realizó un estudio analítico y prospectivo en 15 pacientes con diagnóstico confirmado de linfoma no-Hodgkin y síntomas neurológicos, con citología negativa del líquido cefalorraquídeo, tratados en el servicio de oncología del Instituto Nacional de Oncología y Radiobiología, durante los años 2017 y 2018. El inmunofenotipo fue caracterizado mediante citometría de flujo multiparamétrica. Resultados: El 60,0 por ciento de los pacientes fue del sexo femenino y el 53,4 por ciento mayor de 60 años. Hubo una mortalidad del 26,7 por ciento. Se realizaron 17 inmunofenotipos, el 58,9 por ciento fue normal, el 23,4 por ciento reactivo y el 17,7 por ciento sospechoso de malignidad. La supervivencia global fue mayor en pacientes con líquido cefalorraquídeo con inmunofenotipo normal (HR. 0.04). Conclusiones: La citometría de flujo pudo discriminar células sospechosas de malignidad, en pacientes cuyas citologías fueron negativas. La presencia en el líquido cefalorraquídeo de células atípicas, de pleocitosis y de un índice de linfocito-monocito alto se asoció con una supervivencia global menor(AU)

Introduction: When non-Hodgkin lymphomas infiltrate the central nervous system increases mortality. The diagnosis of this infiltration can be made by the study of cerebrospinal fluid using flow cytometry, with a higher sensitivity than conventional cytology. Objective: To estimate the relationship between the cellular immunophenotype of the cerebrospinal fluid and the overall survival of patients with non-Hodgkin lymphoma and neurological symptoms. Methods: An analytical and prospective study was conducted in 15 patients with confirmed diagnosis of non-Hodgkin lymphoma and neurological symptoms, with negative cytology of the cerebrospinal fluid. Patients cared at Oncology Department of the National Institute of Oncology and Radiobiology, during the years 2017-2018. The immunophenotype was characterized by multiparametric flow cytometry. Results: 60.0 percent of the patients was female and 53.4 percent older than 60 years. There was an overall mortality of 26.7 percent 17 immunophenotypes were found, 58.9 percent of them was normal, 23.4 percent reactive and 17.7 percent suspected of malignancy. Overall survival advantage was obtained in patients with cerebrospinal fluid with normal immunophenotype (HR 0.04). Conclusions: Flow cytometry could discriminate cells suspected of malignancy, in patients whose cytologies were negative. The presence in the cerebrospinal fluid of atypical cells, pleocytosis and a high lymphocyte-monocyte index were associated with a lower overall survival(AU)

OncoHodgk: Aplicación interactiva para el aprendizaje del diagnóstico y tratamiento de los Linfomas / OncoHodgk: Interactive application for learning the diagnosis and treatment of Lymphomas

Introducción: Los linfomas constituyen un grupo de enfermedades malignas, caracterizadas por la proliferación neoplásica del sistema retículoendotelial. Objetivo: Elaborar una aplicación interactiva sobre el diagnóstico y tratamiento de los linfomas para los estudiantes de 6to año de medicina de la Filial de Ciencias Médicas de Bayamo. Diseño Metodológico: La aplicación se elaboró entre febrero y marzo del 2018 en la Filial de Ciencias Médicas de Bayamo. Diseñado con el empleo de las herramientas JClic y Gimpshop 2.8 con licencia GPL/ Linux. Para determinar su efectividad se realizaron encuestas a estudiantes y profesores del centro. El universo estuvo constituido por todos los estudiantes del 6to año de la carrera de Medicina (281 alumnos) y la muestra por los estudiantes seleccionados que realizaron las dos primeras rotaciones por la sala de Medicina Interna (40 estudiantes). El grupo control, estuvo formado por la primera rotación, mientras que el experimental correspondió a la segunda rotación, ambos seleccionados por muestreo aleatorio simple. Resultados: Las mejores calificaciones comprendidas entre 4 y 5 puntos, correspondieron al grupo experimental con 20 estudiantes para el 100 por ciento, mientras que para el control esas calificaciones fueron obtenidas por 15 estudiantes, para un 75 por ciento. Conclusiones: Se constató la efectividad de la aplicación OncoHodgk para el aprendizaje del diagnóstico y tratamiento de los linfomas como alternativa para incrementar el nivel de conocimientos y la motivación en los estudiantes evaluados(AU)

Introduction: Lymphomas constitute a group of malignant diseases, characterized by the neoplastic proliferation of the reticuloendothelial system. Objective: To develop an interactive application about the diagnosis and treatment of lymphomas for the 6th year medical students of the Medical Sciences Branch of Bayamo. Methodological Design: The application was developed between February and March 2018 in the Medical Sciences Branch of Bayamo. It was designed with the use of JClic and Gimpshop 2.8 tools with GPL / Linux license. To determine its effectiveness, surveys were conducted to students and teachers of the center. The universe was constituted by all the students of the 6th year of the Medicine career (281 students) and the sample by the selected students who made the first two rotations through the Internal Medicine room (40 students). The control group was formed by the first rotation, while the experimental group corresponded to the second rotation, both selected by simple random sampling, in a period from February to March 2018, at the University Teaching Hospital Carlos Manuel de Céspedes. Results: The best grades between 4 and 5 points corresponded to the experimental group with 20 students for 100 percent, while in the control group 15 students obtained those grades for 75 percent. Conclusions: The effectiveness of the OncoHodgk application for learning about the diagnosis and treatment of lymphomas was stated(AU)

Hibridación in situ fluorescente (FISH) en el Instituto Nacional de Cancerología (INC) de Colombia. Experiencia de 5 años / Fluorescent in situ hybridization (FISH) in the National Institute of Cancerology (INC) of Colombia. 5 years experience

Resumen Introducción: La hibridación in situ fluorescente (FISH) es una herramienta fundamental en oncopatología para confirmar el diagnóstico de algunas patologías, al igual que determinar el pronóstico y el tratamiento. Objetivo: Describir la experiencia del Instituto Nacional de Cancerología de Colombia (INC) con la técnica de FISH en las diferentes neoplasias hematológicas y tumores sólidos para conocer el comportamiento molecular de nuestra población. Materiales y métodos: Se realizó un estudio descriptivo retrospectivo de todos los resultados de FISH que se han realizado en tumores hematológicos y tumores sólidos en el laboratorio de Genética y Oncología Molecular del INC, entre 2012 y 2016. Resultados: En total se realizaron 1.713 pruebas de FISH, 1.010 (59%) fueron desarrolladas en neoplasias de origen hematolinfoide y 703 (41%) en tumores sólidos, de estos 428 (61%) correspondieron para HER2 de cáncer de seno. En tumores de tejidos blandos fueron evaluadas las sondas MDM2/CDK4, EWSR1, SS18, FUS, CHOP observando positividad en el 10%, el 43%, el 44%, el 20% y el 63%, respectivamente. En cáncer de pulmón se observó positividad en el 12%. Además se realizó estudios para la detección de melanoma y para la detección la codeleción del 1p/19q en gliomas. Discusión: En el INC de Colombia se confirmó la utilidad de la técnica de FISH como complemento en el diagnóstico, el pronóstico y el factor predictivo en el manejo de pacientes con cáncer. Observamos que la prevalencia de algunas pruebas varían de la reportadas en la literatura médica (C-MYC para linfomas, ALK para cáncer de pulmón).

Abstract Introduction: Fluorescent in situ hybridization (FISH) is a fundamental tool in oncopathology to confirm the diagnosis of some pathologies, as well as to determine the prognosis and treatment. Keywords: FISH; Hybridization; Lymphomas; Leukemia; Sarcomas; HER2 Objective: To describe the experience of the FISH in the National Institute of Cancerology of Colombia (INC) in different hematological malignancies and solid tumors to know the molecular behavior of our population. Materials and methods: A retrospective descriptive study was conducted of all the FISH results that have been carried out in the Genetics and Molecular Oncology laboratory of the INC between 2012 and 2016 in hematological tumors and solid tumors. Results: A total of 1713 FISH tests were performed, 1010 (59%) were developed in neoplasms of hematolymphoid origin and 703 (41%) in solid tumors, of these 428 (61%) corresponded to breast cancer (HER2). In soft tissue tumors, MDM2 / CDK4, EWSR1, SS18, FUS, CHOP probes were evaluated, observing positivity in 10%, 43%, 44%, 20% and 63%, respectively. In lung cancer, it has observed positivity in 12%. In addition, studies have been carried out to detect melanoma and to detect the 1p / 19q deletions in gliomas. Discussion: The INC of Colombia confirms the usefulness of the FISH technique as a complement in the diagnosis, prognosis and predictive factor in the management of patients with cancer. We observed that the prevalence of some tests varies from that reported in the medical literature (C-MYC for lymphomas, ALK for lung cancer).

Renal intravascular lymphoma in a dog / Linfoma intravascular renal em um cão

ABSTRACT: A 10-year-old male large mixed breed dog was presented with skin ulcers and fracture on the right hind limb caused by vehicle collision. Given required limb amputation, and as being a shelter senior dog, euthanasia was requested by the owner and a complete post-mortem examination was conducted immediately after death. Gross changes were consistent with marked bilateral nephromegaly. Histopathological examination of the kidneys revealed round cells filling blood vessels. Immunohistochemically, the round cells were positive for CD3 antibody. Based on these findings, in absence of involvement of the bone marrow and peripheral blood, and inexistence of primary extravascular masses, the tumor was classified as T-cell intravascular lymphoma. To the author's knowledge, this is the first report describing intravascular lymphoma involving the kidneys alone in a dog.

RESUMO: Um canino, macho, de 10 anos, sem raça definida (SRD), e grande porte, chegou para atendimento apresentando fratura em membro pélvico direito devido a atropelamento por veículo automotivo. Adicionalmente, foram observadas úlceras cutâneas ao nível da fratura. Devido à necessidade de amputação do membro e, por ser um cão idoso, o proprietário optou pela eutanásia, realizando-se necropsia imediatamente após a morte do paciente. Os achados macroscópicos foram consistentes com acentuada nefromegalia bilateral. A avaliação histopatológica dos rins revelou células redondas neoplásicas obliterando vasos sanguíneos. Imunohistoquimicamente, essas células foram positivas para CD3. Baseando-se nos achados histopatológicos, na ausência de envolvimento da medula óssea e do sangue periférico e, na inexistência de massas primárias extravasculares, o tumor foi classificado como linfoma intravascular de células T. Possivelmente, este é o primeiro relato de linfoma intravascular envolvendo unicamente os rins de um cão.

The First Year of the AEVD Primary Cutaneous Lymphoma Registry. / Registro de linfomas cutáneos primarios de la AEDV: primer año de funcionamiento.

BACKGROUND AND OBJECTIVE: Primary cutaneous lymphomas are uncommon. This article describes the Primary Cutaneous Lymphoma Registry of the Spanish Academy of Dermatology and Venereology (AEDV) and reports on the results from the first year. PATIENTS AND METHODS: Disease registry for patients with primary cutaneous lymphoma. The participating hospitals prospectively recorded data on diagnosis, treatment, tests, and disease stage for all patients with primary cutaneous lymphoma. A descriptive analysis was performed. RESULTS: In December 2017, the registry contained data on 639 patients (60% male) from 16 university hospitals. The most common diagnoses, in order of frequency, were mycosis fungoides/Sézary syndrome (MF/SS) (348 cases, 55%), primary cutaneous B-cell lymphoma (CBCL) (184 cases, 29%), primary cutaneous CD30+ T-cell lymphoproliferative disorder (CD30+ CLPD) (70 cases, 11%), and other types of T-cell lymphoma (37 cases, 5%). In total, 105 (16.5%) of the cases recorded were incident cases. The most common diagnosis in the MF/SS group was classic MF (77.3%). Half of the patients with MF had stage IA disease when diagnosed, and the majority were either in partial remission (32.5%) or had stable disease (33.1%). The most widely used treatments were topical corticosteroids (90.8%) and phototherapy. The most common form of primary CBCL was marginal zone lymphoma (50%). Almost all of the patients had cutaneous involvement only and nearly half had stage T1a disease. Most (76.1%) were in complete remission. The main treatments were surgery (55.4%) and radiotherapy (41.9%). The most common diagnosis in patients with CD30+ CLPD was lymphomatoid papulosis (68.8%). Most of the patients (31.4%) had stage T3b disease and half were in complete remission. The most common treatments were topical corticosteroids (68.8%) and systemic chemotherapy (32.9%). CONCLUSION: The characteristics of patients with primary cutaneous lymphoma in Spain do not differ from those described in other series in the literature. The registry will facilitate clinical research by the AEDV's lymphoma group.

Presencia del marcador lmp-1 del virus epstein-barr en linfomas de caninos / Presence of lmp-1 protein of epstein-barr virus in lymphomas of canine origin

RESUMEN El virus Epstein-Barr (EBV) es un virus de alta prevalencia en humanos que se asocia con tumores de la línea linfoide B. En caninos se dispone de pocos reportes sobre la presencia del EBV y su rol en esta especie. El objetivo del presente estudio fue determinar la presencia de la proteína latente de membrana del EBV (LMP-1) en tejidos obtenidos de 20 linfomas de caninos cuyo diagnóstico se había realizado durante un periodo de 10 años, entre 2004 y 2014. Los linfomas se reclasificaron mediante las nuevas clasificaciones histopatológicas para linfomas y se sometieron a inmunohistoquímica (IHQ) con los anticuerpos anti-CD79a, anti-CD3, anticuerpos específicos para linfocitos B y T, además de un anti-LMP-1 como marcador de la presencia del EBV. Se encontró que el linfoma más común fue el linfoma nodal de zona T con un 75% de los casos. Al realizar la inmunomarcación se encontraron 18 casos positivos a CD3, 2 casos positivos a CD79a y 6 casos positivos a LMP-1, lo que representa el 30% de positividad del EBV en linfomas. El análisis Ji cuadrado demostró significancia estadística entre la presencia del virus y la presencia del linfoma lo que sugiere, no solamente que el virus está circulando en la población canina, sino que además puede tener relación con la ocurrencia de esta neoplasia. Con relación a las variables demográficas, sólo en la raza Golden Retriever se demostró relación con la presencia del linfoma, pero no con la presencia del virus.

ABSTRACT Epstein Barr virus (EBV) is a human high prevalent virus associated with lymphoid B cells tumors development. In canines, few reports have been published regarding the presence of the virus in dogs but its role in this species remains unclear. The aim of this study was to determine the presence of LMP-1 protein of EBV in 20 canine lymphomas tissues which were previously diagnosed in a period of time between 2004 -2014. Lymphomas were reclassified in accordance with the new histopathological classifications for lymphomas and were stained by IHQ with anti-CD79a, anti-CD3 and anti-LMP-1; in addition, specific antibodies for B lymphocytes, T lymphocytes and EBV biomarker, respectively. It was found that the most common lymphoma was T-zone lymphoma in 75% of the cases of the study. The distribution of the cases regarding the immunostaining was: 18 positive cases with anti-CD3, 2 positive cases with anti-CD79a and 6 positive cases with anti- LMP-1. Positive cases of LMP-1 as a biomarker of the presence of EBV corresponded to the 30% of the cases of the study. Chi-square test showed statistical significance between the presence of the virus and the presence of lymphomas, which suggests not only that the virus is circulating in the canine population but also that could have implications in the development of the disease. Regarding demographic parameters, only the Golden Retriever breed showed a relationship with the presence of lymphoma, but not with the presence of the virus.

Linfoma de burkitt: a propósito de un caso / Burkitt lymphoma: a case report

Resumen:Los linfomas son un conjunto de enfermedades neoplásicas que se originan en células del sistema inmunitario, y que afectan específicamente a las células del sistema linfopoyético, que es el encargado de producir los linfocitos. Son considerados como la tercera enfermedad maligna más frecuente de la niñez, representan aproximadamente, el 12% de los casos nuevos de cáncer diagnosticados en los menores de 15 años. De éstos, alrededor de 40% son linfomas de Burkitt.

Abstract:Lymphomas are a set of neoplastic diseases that originate in cells of the immune system and that specifically affect cells lymphopoietic system, which is responsible for producing lymphocytes. They are regarded as the third most common malignant disease of childhood, representing approximately 12% of new cases of cancer diagnosed in children under 15 years. Of these, about 40 % are Burkitt lymphomas.

Conjunctival lymphoma in right eye: Case report. / Linfoma conjuntival de ojo derecho: a propósito de un caso.

CASE REPORT: A 43-year-old woman presented with a salmon-coloured patch of 0.7mm diameter in the right eye that extended into the lower fornix in the bulbar and tarsal conjunctiva, with irregular edges, and highly vascularised. Incisional biopsy was performed, showing it to be a low-grade conjunctival non-Hodgkin B cell lymphoma (or a mucosa associated lymphoid tissue [MALT] lymphoma). DISCUSSION: The lesion remained stable for 24 months of follow-up, when a relapse of the condition occurred, producing an enlargement of the initial lesion. The definitive diagnosis is made by biopsy of the affected tissue and histopathologic study.