Linfoma no Hodgkin localizado en el tejido mamario. presentación de un caso / Non-Hodgkin lymphoma located in the breast tissue. presentation of a case

Se presentó el caso de una adolescente de 12 años de edad que acudió a consulta externa de Cirugía del Hospital Pediátrico Docente "Gral. Pedro Agustín Pérez", de Guantánamo; la misma presentó aumento de volumen a nivel del cuadrante superior externo de la mama izquierda, no doloroso. Se palpó tumoración de bordes irregulares, o sea mal definidos, de 3 cm de diámetro aproximadamente, sin signos de inflamación. Se realizó biopsia por aspiración con aguja fina y se informó el diagnóstico de fibroadenoma de mama. La exéresis del nódulo y el estudio histopatológico demostraron el diagnóstico de linfoma no Hodgkin de alto grado de malignidad y se procedió a la atención multidisciplinaria. Al momento del reporte la paciente continúa con quimioterapia y evoluciona satisfactoriamente. Se revisó la literatura médica sobre el tema y se expusieron sus bases esenciales para la familiarización de los médicos generales(AU)

A 12 years old young female presented to the surgical outpatient clinic at the Hospital Pediátrico Docente "Gral. Pedro Agustín Pérez" in Guantanamo. Patient showed swelling in the upper right breast, with no pain. Examination revealed irregular edges (with approximately 3 cm in diameter) and no swollen tissue. Fine-needle aspiration biopsy was performed and breast fibroadenoma was diagnosed. Node exeresis and histopathological study both showed a high risk malignant non-Hodgkin's lymphoma, and a multidisciplinary care team assumed the case. At the time of the report, the patient is still under chemotherapy and prognosis evolves positively. The medical literature on the subject was put on review and a layout of the essentials to familiarize the general practitioners with the topic was set(AU)

Linfoma no Hodgkin localizado en el tejido mamario. Presentación de un caso / Non-Hodgkin lymphoma located in the breast tissue. Presentation of a case

RESUMEN Se presentó el caso de una adolescente de 12 años de edad que acudió a consulta externa de Cirugía del Hospital Pediátrico Docente Gral. Pedro Agustín Pérez, de Guantánamo; la misma presentó aumento de volumen a nivel del cuadrante superior externo de la mama izquierda, no doloroso. Se palpó tumoración de bordes irregulares, o sea mal definidos, de 3 cm de diámetro aproximadamente, sin signos de inflamación. Se realizó biopsia por aspiración con aguja fina y se informó el diagnóstico de fibroadenoma de mama. La exéresis del nódulo y el estudio histopatológico demostraron el diagnóstico de linfoma no Hodgkin de alto grado de malignidad y se procedió a la atención multidisciplinaria. Al momento del reporte la paciente continúa con quimioterapia y evoluciona satisfactoriamente. Se revisó la literatura médica sobre el tema y se expusieron sus bases esenciales para la familiarización de los médicos generales.

ABSTRACT A 12 years old young female presented to the surgical outpatient clinic at the Hospital Pediátrico Docente Gral. Pedro Agustín Pérez in Guantanamo. Patient showed swelling in the upper right breast, with no pain. Examination revealed irregular edges (with approximately 3 cm in diameter) and no swollen tissue. Fine-needle aspiration biopsy was performed and breast fibroadenoma was diagnosed. Node exeresis and histopathological study both showed a high risk malignant non-Hodgkin's lymphoma, and a multidisciplinary care team assumed the case. At the time of the report, the patient is still under chemotherapy and prognosis evolves positively. The medical literature on the subject was put on review and a layout of the essentials to familiarize the general practitioners with the topic was set.

Linfoma no Hodgkin primario testicular / Non hodgkin primary testicular limphoma

Los linfomas constituyen un grupo diverso de neoplasias malignas, que se originan del sistema linfático(1). Aproximadamente el 85% se agrupan dentro de los denominados Linfomas No Hodgkin (LNH). Los LNH constituyen un numeroso grupo de linfomas derivados de la transformación neoplásica de los linfocitos B, T y Células NK, provenientes de diversas zonas que comprometen los folículos linfáticos en los nódulos linfáticos o en el sistema linfoide extranodal(2). Las localizaciones extra ganglionares primarias representan más de 10% de los casos y los sitios más frecuentes comprenden estómago, intestino delgado, orofaringe, piel, hígado y cerebro; además de otras bastante raras como ano, corazón, músculos y encías. Los linfomas testiculares primarios son muy raros, representan aproximadamente el 5% de todas las neoplasias testiculares malignas, menos del 1% de los LNH y el 4% de los LNH extra nodales(3). Esta neoplasia constituye el tumor maligno testicular más frecuente en sujetos ancianos. La presentación clínica típica es una masa testicular unilateral acompañada en ocasiones de hidrocele y dolor agudo escrotal. A continuación presentamos un caso que requirió de la participación de múltiples especialidades de nuestra institución para un manejo conjunto y finalmente el paciente falleció(AU)

The lymphomas constitute a diverse group of malignant neoplasms, which originate from the lymphatic system(1). Approximately 85% are grouped into the so-called Non-Hodgkin's Lymphomas (NHL). NHL are a large group of lymphomas derived from the neoplastic transformation of B, T and NK cells of different areas with involvement of lymphatic follicles in the lymph nodes or the extranodal lymphoid system(2). The primary extraganglionic sites represent more than 10% of the cases and the most frequent sites include stomach, small intestine, oropharynx, skin, liver and brain; others, quite rare, are anus, heart, muscles and gums.Primary testicular lymphomas are extremely rare, accounting for approximately 5% of all malignant testicular neoplasms, less than 1% of NHL and 4% of extranodal NHL(3). This neoplasm constitutes the most frequent testicular malignant tumor in elderly subjects. The typical clinical presentation is a unilateral testicular mass, sometimes accompanied by hydrocele and acute scrotal pain. We present a case which required the participation of multiple specialties of our institution for management but the patient finally died(AU)

Linfoma primario del intestino delgado: reporte de un caso y revisión de la literatura / Primary Lymphoma in the Small Intestine: A Case Report and Literature Review

El tracto gastrointestinal es el sitio más frecuente de presentación del linfoma no Hodgkin (LNH) extraganglionar. Sin embargo, los linfomas primarios del tracto gastrointestinal son tumores raros y es mucho más frecuente la afectación de este tracto de manera secundaria en el curso de la enfermedad. Representan del 1% al 4% de los tumores malignos del tracto gastrointestinal, aunque tienen un curso, manejo y pronóstico muy diferente de los adenocarcinomas, por lo que es importante conocerlos y tenerlos en mente como un diagnóstico diferencial posible en el ejercicio clínico diario. Afectan más a los adultos jóvenes, con una mayor frecuencia en hombres (1). Reportamos el caso de una mujer de 47 años de edad con linfoma primario del intestino delgado, diagnosticado luego de múltiples consultas por síntomas abdominales y revisamos la literatura al respecto

The gastrointestinal tract is the most frequent site of non-Hodgkin’s lymphoma (NH) outside of the lymph nodes themselves. This tract is much more frequently compromised by tumors secondary to primary disease elsewhere in the body than by primary lymphomas of the gastrointestinal tract itself which are rare. They account for only one to four percent of malignant tumors of the gastrointestinal tract. Their development and prognoses are quite different from those of adenocarcinomas, hence their management must differ as well. It is important to understand them and keep them in mind in differential diagnosis in daily clinical practice. Young adults are most frequently affected, and men are more frequently affected than are women. We review the literature and report the case of a 47 year old woman with primary small bowel lymphoma that was diagnosed after several consultations due to abdominal symptoms

Human umbilical cord-drived mesenchymal stem cells as vehicles of CD20 specific-TRAIL fusion protein against non-Hodgkin’ s lymphoma / 中国免疫学杂志

Objective:To study the therapeutic effect of a novel double-target system,in which human umbilical cord-derived MSCs were used as vehicles to deliver fusion protein scFvCD20:sTRAIL to non-Hodgkin ’ s lymphoma. Methods: The traditional methods in molecular biology were used to construct lentivirus expression vectors pLenR. scFvCD20: sTRAIL and contrast vectors. Human umbilical cord-derived MSCs ( HUMSCs ) were labeled with the copGFP by transducing with pseudo viral particles which had been packaged in 293T cells with four plasmid-lentivirus packaging system. Fusion protein scFvCD20:sTRAIL were secreted from MSC. scFvCD20:sTRAIL after that HUMSCs were infected by pseudo viral particles. CCK8 assay was applied to detect the antigen-restricted cell death induced by scFvCD20:sTRAIL in CD20-positive BJAB and Raji cells as well as CD20-negtive Jurkat cells and human normal peripheral blood mononuclear cells (PBMCs). To evaluate the therapeutic effect of MSC. scFvCD20:sTRAIL in vivo,ge-netically modified HUMSCs were intravenously injected into tumor-bearing mice with BJAB cells. The volume of tumor was measured every three days, and the inhibition ratio of tumor was calculated according to tumor volume. Results: Lentivirus expression vectors pLenR. scFvCD20:sTRAIL, pLenR. ISZ:sTRAIL, pLenR. scFvCD20 and pLenR. CopGFP were successfully constructed and these constructs could be expressed stably in HUMSCs by lentivirus transduction. scFvCD20:sTRAIL fusion protein produced a potent inhibition of cell proliferation in CD20-positive BJAB cells,moderate inhibition of the growth of Raji cells,and weak inhibition in CD20-negtive Jurkat cells when compared with ISZ-sTRAIL treatment,and it had no effect on normal human peripheral blood mononuclear cells (PBMCs). The MSC. scFvCD20:sTRAIL treatment significantly inhibited the tumor growth when compared with those treated with MSC. ISZ-sTRAIL. Conclusion: A double-target therapeutic system is well established, in which HUMSCs migrated to tumor site, secreted a novel fusion protein scFvCD20:sTRAIL,and thus locally concentrated scFvCD20:sTRAIL extended antigen-restricted anti-tumor activity. The engineered HUMSCs secreting scFvCD20:sTRAIL showed potent effect on inhibiting tumor growth in BJAB lymphoma malignancy,which may play an essential role in the clinical research .

Factores que participan en la interacción huésped-agente patógeno en el riesgo de Linfoma de Hodgkin inducido por el virus Epstein Barr / Factors involved in host-pathogen interaction for the risk of Hodgkin lymphoma induced by Epstein Barr virus

El linfoma de Hodgkin (LH) es una neoplasia del sistema linfático. La incidencia mundial anual del LH es de 3-10/100,000 habitantes. El mecanismo mediante el cual se lleva a cabo la transformación celular no es completamente claro; sin embargo, algunas evidencias parecen indicar que ciertos virus oncogénicos como el virus Epstein Barr (VEB), pueden tener alto impacto en la patogénesis de la linfoproliferación. También algunos factores genéticos y ambientales pueden estar involucrados, pues se ha encontrado una alta incidencia de casos de LH entre individuos de una misma familia que comparten características genéticas y conviven en un mismo ambiente. En México se han realizado estudios encaminados a conocer la prevalencia del VEB en pacientes con LH y se ha encontrado la presencia de este virus hasta en el 64,2%. El VEB ha sido detectado en las Células Reed Sternberg (CRS) y en Células de Hodgkin (CH) en el 50% de los casos de LH clásico. No se ha dado hasta ahora una explicación satisfactoria, pero se ha propuesto que la variabilidad geográfica y la variabilidad inmunológica desempeñan un papel determinante en la positividad del VEB en LH. A pesar de los avances que hasta ahora se tienen, no existen suficientes evidencias que permitan establecer una clara asociación entre los factores del huésped, el medio ambiente y el agente patógeno en el riesgo de la linfoproliferación que conduce al desarrollo de LH. La presente revisión tiene como objetivo analizar algunos de los factores de riesgo que influyen durante la interacción huésped, agente patógeno y medio ambiente en la etiología del LH.

Hodgkin lymphoma (HL) is a neoplasm characterized by malignant cells called Reed Sternberg and Hodgkin’s cells in the lymphatic system. Such cells comprise 1% of the tumor while the remainder is made up of lymphocytes, histiocytes, eosinophils and plasma non-neoplastic cells. The annual global incidence of HL is 3-10/100,000 inhabitants and is most commonly found in young adults. The mechanism by which cell transformation is accomplished is not entirely clear; however, some evidences suggest that oncogenic viruses like the Epstein Barr virus (EBV) may have a high impact on the pathogenesis of lymphoproliferation. Genetic and environmental factors could be involved, since it has been found a high incidence of HL among members of the same family. In Mexico, there have been studies to determine the prevalence of EBV in patients with HL and found the presence of this virus in up to 64.2% of the cases. EBV has been detected in the Reed Sternberg cells and Hodgkin cells in 50% of cases of classical HL. There is not a satisfactory explanation for this, but it has been proposed that geographic and immunological variabilities play a role in the positivity of EBV in HL. However, despite recent advances in the field, there is insufficient evidence to show a clear association between host factors, environment and pathogens, and the risk of lymphoproliferation leading to the development of HL. This review aims to give an overview about the risk factors that influence the interaction of host, pathogens and environment in the etiology of HL.

Linfoma no Hodgkin y leucemia mieloide crónica metacrónica en una paciente: reporte de un caso / Non Hodgkin´s lymphoma and metachronus chronic myeloid leukemia in a patient: case report

INTRODUCCIÓN: Los linfomas no Hodgkin son neoplasias linfáticas de incidencia creciente en el mundo y que en Chile representan la séptima causa de muerte por cáncer. El tratamiento con quimioterapia de los linfomas de alto grado de malignidad ha significado que globalmente se logren sobrevidas de 60 por ciento a cinco años. La ocurrencia de segundas neoplasias en pacientes con linfomas tratados es aproximadamente un 7 por ciento, correspondiendo la mayoría a tumores sólidos de pulmón, mama y leucemias agudas. No se ha descrito la leucemia mieloide crónica como complicación del tratamiento o forma secundaria en pacientes tratados por linfoma. PRESENTACIÓN DEL CASO: Paciente que un año y medio después de ser tratada de una recaída tardía de un Linfoma no Hodgkin de alto grado, avanzado, presenta leucemia mieloide crónica confirmada por cariograma y reacción de polimerasa en cadena. Recibe tratamiento con Imatinib con respuesta citogenética completa. DISCUSIÓN: Revisada la literatura no se encontró reportado otro caso similar. Está descrita la asociación entre ambas entidades, pero siempre el linfoma sigue o se presenta simultáneamente con la leucemia mieloide crónica. Esto podría tratarse de diferentes formas de expresión de una alteración de la stem cell pluripotente o bien de una ocurrencia al azar en una persona con defecto en los mecanismos antioncogénicos.

INTRODUCTION: Non-Hodgkin´s lymphoma is lymphatic neoplasms with increasing incidence in the world and in Chile represents the 7th cause of cancer death. Chemotherapy treatment of lymphomas of high malignancy has meant that overall survivals are prolonged in approximately 60 percent of all patients. The occurrence of second malignancies in patients after treatment of lymphoma is approximately 7 percent, beeing mostly solid tumors of lung, breast and acute leukemias. It has not been reported chronic myeloid leukemia as a complication of treatment or secondarily in patients treated of lymphoma. CASE REPORT: Patient that one and a half year after treatment of a late relapse of non-Hodgkin lymphoma of high grade, advanced, presents a chronic myeloid leukemia confirmed by karyotype and by polymerase chain reaction. After treatment with Imatinib the patient achieved a complete cytogenetic response. DISCUSSION: Review of the literature found no similar case report. It describes the association between both entities but lymphoma always following or occurring simultaneously with chronic myeloid leukemia. This could be different forms of expression of an alteration of the pluripotent stem cell or a random occurrence in a person with antioncogénicos defect mechanisms.

Burkitt's lymphoma: clinic progression and prognosis. Two different cases reports in young patients / Linfoma de Burkitt: evolução clínica e prognóstico. Relato de dois casos diferentes em pacientes jovens

Purpose: Burkitt's lymphoma is one of the fastest growing malignancies in the pediatric population. It is a high-grade B-cell non-Hodgkin's lymphoma with endemic, sporadic and human immunodeficiency-associated subtypes. The African, or endemic, variant usually involves the maxilla and other facial bones, while head and neck manifestations in sporadic Burkitt's lymphoma are rare. Case description: Two cases of oral manifestations of Burkitt's lymphoma are described on the right jaw in young patients, which had different clinical evolution and prognosis.

Objetivo: O linfoma de Burkitt é uma das mais importantes neoplasias de crescimento rápido na população pediatra. É um linfoma Não-Hodgkin primário de células B subdividido em endêmico, esporádico ou associado a imunodeficiência humana. O tipo africano (endêmico), quando em cabeça e pescoço, envolve geralmente os maxilares e outros ossos faciais, enquanto que o envolvimento facial da forma esporádica é bem raro. Descrição do caso: São descritos dois casos intra-bucais de linfomas de Burkitt envolvendo maxila direita em pacientes jovens, os quais apresentaram evolução clínica e prognósticos diferentes.

Mortalidade por linfoma não Hodgkin em capitais da região sudeste e sobrevida de pacientes submetidos a transplante no Instituto Nacional do Câncer / Mortality from non-Hodgkin's capital in the Southeast and survival of transplant patients at National Cancer Institute

A mortalidade por linfoma não Hodgkin vem diminuindo em vários países, porém, para o Brasil, as estimativas apontam um crescimento em ambos os sexos. O objetivo deste estudo foi analisar a tendência da mortalidade por linfoma não Hodgkin em indivíduos com 20 ou mais anos, nas capitais da região Sudeste, entre 1980 e 2007. Utilizou-se como fonte de dados o Sistema de Informação sobre Mortalidade e o DATASUS. A tendência das taxas de mortalidade por linfoma não Hodgkin por faixas etárias foi analisada por meio de modelos de regressão polinomial. Foi observada tendência linear de incremento em Belo Horizonte e São Paulo para faixa etária de 60 ou mais anos. Ao analisar de forma separada os períodos 1980-1995 e 1996-2007, só se observou tendência de incremento no período inicial. Os resultados sugerem que o incremento observado entre 1980-2007 poderia ser resultante do crescimento das taxas entre 1980-95, já que, no último período, não foram observadas tendências estatisticamente significativas nessas cidades. A partir da década de 90, o transplante de células-tronco hematopoiéticas (TCTH) passou a ser utilizado de forma mais consistente no tratamento de pacientes com linfoma não-Hodgkin (LNH) e, entre as possíveis explicações para a redução da mortalidade por essa neoplasia, observada nas últimas décadas em diferentes países, encontram-se os avanços no seu tratamento. Realizou-se um estudo descritivo de sobrevida em uma coorte histórica de pacientes com LNH, de 18 ou mais anos de idade, submetidos ao primeiro TCTH em um hospital de referência em oncologia no Rio de Janeiro, entre janeiro de 1997 e maio de 2009. As informações do estudo foram obtidas através da análise dos prontuários médicos, utilizando-se instrumento elaborado especificamente para essa finalidade...

Mortality from non-Hodgkin’s lymphoma has decreased in many countries although for Brazil estimates indicate a growth in both sexes. The aim of this study was to analyze trends in mortality from non- Hodgkin’s lymphoma in individuals with 20 or more years in the capitals of the southeastern region ofBrazil between 1980 and 2007. Data were obtained through the National Information System on Mortalityand from the Brazilian Institute of Geography and Statistics. Trends of mortality rates for non-Hodgkin’slymphoma by age were analyzed by polynomial regression models. A linear trend of increment of the mortality was observed in Belo Horizonte and Sao Paulo for the age 60 years or older. When analyzed separately in two periods 1980-1995 and 1996-2007, a trend of increase was only observed in the initial period. These results suggest that the increment observed between 1980 and 2007 could be related toincreased mortality rates between 1980-95, as there were no statistically significant trends in these cities inthe last period. Since the 90's, hematopoietic stem cell transplantation (HSCT) is being used more consistently in the treatment of patients with non-Hodgkin lymphoma (NHL) and these advances are among the possibleexplanations for the reduction of mortality from this neoplasia, observed in recent decades in different countries. We conducted a descriptive study of survival in a historical cohort of patients with NHL, 18 or more years old, who underwent a first HSCT in a referral hospital in Rio de Janeiro, between January 1997 and May 2009. Study information was obtained through analysis of medical records, using aninstrument developed specifically for this purpose. We considered as the study’s outcomes: overall survival (in months), estimated from the date of transplantation until death; and disease-free survival (in months), estimated from the date of transplant until evidence of disease after transplantation...

Associação rara de duas neoplasias malignas em paciente portadora de febre de origem obscura: relato de caso / Rare association between two types of cancer in patient with fever of unknown origin: case report

JUSTIFICATIVA E OBJETIVOS: A febre de origem obscura é uma entidade presente no dia-a-dia do clínico, muitas vezes de difícil diagnóstico e tratamento. O objetivo deste estudo foi ressaltar a importância de pesquisar um segundo tumor maligno como causa de febre de origem obscura em pacientes portadores de neoplasia maligna de mama. RELATO DO CASO: Paciente do sexo feminino, 58 anos, portadora de câncer de mama submetida a tratamento multimodal, que evoluiu com febre de origem obscura decorrente do aparecimento de segundo tumor primário, um linfoma de alto grau, acompanhado por manifestações paraneoplásicas cutâneas e neurológicas, assim como importante síndrome de resposta inflamatória sistêmica não relacionada à infecção intercorrente. CONCLUSÃO: O aspecto extremamente incomum da associação foi o de linfoma não-Hodgkin, este representando o segundo tumor primário e adenocarcinoma primário da mama.

BACKGROUND AND OBJECTIVES: Fever of unknownorigin is a common entity present on the clinician's work dayand, usually, difficult to diagnose. The aim of this paper was to enhance the importance of look after a second cancer in a patient with fever of unknown origin and breast cancer. CASE REPORT: A female patient, 58-year-old with breast cancer, who was being submitted to multimodal treatment, developed a fever of unknown origin, due to the appearance of a second primary tumor, a high grade malignant lymphoma, accompanied by cutaneous and neurologic paraneoplastic manifestations, as well as a significant systemic inflammatory response syndrome, unrelated to infection. CONCLUSION: The extremely uncommon aspect of the association was of a non-Hodgkin lymphoma, representing a second primary tumor, and a breast adenocarcinoma.

Linfomas não hodgkin em crianças e adolescentes um estudo Descritivo no estado da Bahia-Brasil / Non - Hodgkin lymphoma in children and adolescents: a descriptive Study in the state of Bahia - Brazil

O objetivo desta pesquisa foi investigar as características sócio-demográficas e clínico-patológicas dos linfomas não Hodgkin LNH em crianças e adolescentes no estado da Bahia. Do ponto de vista da metodologia, trata se de estudo descritivo com dados secundários de 111 pacientes com LNH, procedentes de dois centros de referência. Critérios de inclusão idade 20 anos, residência na Bahia e admissão no período de janeiro de 2000 a dezembro de 2005, com diagnóstico anátomo patológico de LNH. Obtiveram se os seguintes resultados: dos 111 pacientes, 76 68,5% presentaram idade entre cinco e 14 anos, com uma média de 8,6 4,3 anos. Linfomas de Burkitt LB predominaram 56,8%seguidos pelos linfomas linfo blásticos e linfomas de grandes células, cada um com 23 casos 20,7%. Sítio primário do tumor predominante foi abdominal 57,7 %, seguido por cabeça e pescoço 19,8%, mediastino 16,2% e outros 6,3%. Manifestações clínicas mais freqüentes nos pacientes com LB foram: dor abdominal 39,7%, aumento do volume abdominal 20,6%e presença de massa no abdome 12,7%. Quase 41% dos pacientes com LB apresentavam sintomas há mais de um mês. O valor médio da enzima lactato desidrogenase foi 1.896 4.277,6 U/L nos pacientes com LB. Os resultados apontam as características quepredominaram em pacientes com LNH pediátricos. Deve-se sempre cogitar sobre a presença dessa afecção como diagnóstico diferencial quando tais características estiverem presentes.

To investigate socio-demographic and clinical-pathological characteristics of the non-Hodgkin?s lymphoma NHL in children and adolescents in the State of Bahia. It is a descriptive study with secondary data from 111 patients with NHL, coming from two reference health centers. Criteria for inclusion: age 20 years, resident in Bahia, and admission from January 2000 to December 2005, with anatomic pathological diagnosis of NHL. Among the 111 patients, 76 68.5% were aged 5 14 years, with a mean of 8.6 4.3 years. The Burkitt lymphoma BL prevailed 56.8%, followed by lymphoblastic lymphoma LL and large cell lymphoma LCL, each with 23 cases 20.7%. Abdominal region was the dominant site 57.7%, followed by head and neck 19.8%, mediastinum 16.2% and other sites 6.3%. The most frequent clinical manifestations in patients with BL were: abdominal pain 39.7%, increase in the abdominal volume 20.6% and presence of mass in the abdomen 12.7%. Approximately 41% of patients with BL had symptoms one month before diagnosis. Average level of serum lactate dehydrogenase enzyme in patients with BL was 1,896 4,277.6 U L. These results show the characteristics that prevailed in pediatric patients with NHL. So, this disease should be thought of as differential diagnosis when these characteristics are present.

Associação de ictiose adquirida, linfoma de Hodgkin e Hepatite B / Combination of acquired ichthyosis, Hodgkin’s lymphoma and Hepatitis B

Ictiose adquirida é um distúrbio raro da queratinização, associado a diversas doenças, como neoplasias. Aqui relatamos o caso de uma paciente que se apresentou com ictiose de início recente e no decorrer da investigação foi descoberta hepatite B crônica e linfoma de Hodgkin. Não encontramos relatos da associação destas três entidades na literatura.

Acquired Ichthyosis is a rare disorder of keratinization associated with several diseases such as neoplasias. Here we report the case a patient presenting with ichthyosis of recent onset, and along the investigation chronic hepatitis B and Hodgkin lymphoma were diagnosed. No previous report of the combination of these three entities was found in the literature.

Linfoma renal primario en paciente con gammapatía monoclonal IgM / Primary renal lymphoma in a patient with IgM monoclonal gammapathy

Son infrecuentes los casos de linfoma renal primario, ya que la afectación renal por un proceso linfoproliferativo es, por lo general, secundaria a una enfermedad sistémica. Presentamos el caso de una paciente mujer de 48 años que acude por dolor lumbar y masa abdominal. Después de realizar estudios (TC), se práctica nefrectomía cuyo resultado anatomopatológico fue de linfoma no-hodking B primario renal. Asimismo el paciente presentaba una gammapatía monoclonal IgM asociada, por lo que precisó tratamiento quimioterápico sistémico. Realizamos una revisión bibliográfica centrándonos en los criterios diagnósticos y terapéuticos actuales.

Reports on primary renal lymphoma are scarce in the urological literature, the most part f them are secondary on a lymphomatous infiltration of the kidneys. We report the case of a 48 year old women with lumbar pain and adominal mass. After radiological studies (CT), we practise nephrectomy with a pathological result of a non-hodking B primary lymphoma. The patient present a IgM monoclonal gammapathy who need complementary treatment with chemotherapy. A literature review on currently recommended diagnostic and treatment practices in presented.

Tratamento do linfoma de Hodgkin após falha do transplante autólogo / Treatment of Hodgkin's lymphoma after failure of autologous stem cell transplant

O linfoma de Hodgkin (LH) é uma neoplasia do tecido linfóide de excelente prognóstico, porém, aproximadamente 15 por cento dos pacientes em estádios precoces e 35 por cento dos em estádios avançados progridem após o tratamento inicial. O transplante autólogo de medula óssea ou de células-tronco periféricas (ATMO) é o tratamento de escolha nesses casos. Nosso estudo tem como objetivo avaliar o tipo de tratamento utilizado, a taxa de resposta e a sobrevida de pacientes recidivados ou refratários ao ATMO. De 38 pacientes com LH submetidos a ATMO entre abril de 1996 e novembro de 2005, foram avaliados 17 que apresentaram recidiva/refratariedade ao ATMO. Nesses casos, o tratamento de resgate foi individualizado, a depender das condições clínicas de cada um, sendo constituído usualmente de drogas citotóxicas não utilizadas previamente. Após o ATMO, dez (59 por cento) dos 17 pacientes obtiveram remissão completa, um (6 por cento) remissão parcial e seis (35 por cento) foram refratários. Em 14 dos 17 pacientes foi instituída quimioterapia de resgate com diversos esquemas no momento da recidiva/refratariedade após ATMO; um paciente foi tratado com radioterapia exclusiva e dois foram a óbito antes de qualquer terapia. Observamos uma taxa de resposta global de 57,4 por cento (IC95 por cento: 23,2 - 90,7 por cento). A mediana da sobrevida livre de progressão foi de 19 meses e a mediana de sobrevida global foi de 32 meses. Apesar do LH recidivado/refratário ao ATMO não ser curável com os quimioterápicos atualmente disponíveis, os pacientes apresentaram longa sobrevida, com freqüentes exacerbações da doença.

Hodgkin's lymphoma (HL) is a lymphoid malignancy with excellent prognosis, however nearly 15 percent of the patients in early stages and 35 percent in advanced stages have progressive disease after initial treatment. Autologous bone marrow or hematopoietic stem cell transplantation (ABMT) are the treatments of choice in these cases. This report presents the therapeutic approach and the outcome of HL patients who experience relapse after or are refractory to ABMT. Of 38 patients with LH who underwent ABMT between April 1996 and November 2005, 17 presented with relapsed/refractory disease and were included in this analysis. In these cases, the choice of rescue therapy varied upon the clinical conditions of each patient and was based on previously unused chemotherapy agents. After ABMT, 10 (59 percent) of the 17 patients were in complete remission, one (6 percent) in partial remission and six (35 percent) were refractory. Fourteen of the 17 patients received different schemes of rescue therapy at the time of ABMT failure, one patient was treated exclusively with radiotherapy and two died before any treatment. We observed an overall response rate of 57.4 percent (95 percent CI: 23.2 - 90.7 percent). The median progression-free survival was 19 months and the median overall survival was 32 months. Despite ABTM, relapsed/refractory LH can not be cured with currently available chemotherapeutic agents, the patients had long survival times with frequent exacerbations of the diseas.

Localização no calcâneo de linfoma não-Hodgkin B de grandes células primário do osso: relato de caso e revisão de literatura / Calcaneous involvement by primary large B-cell non-Hodgkin's lymphoma of bone: a case report and literature review

Descreve-se, neste artigo, um caso raro de linfoma não-Hodgkin B de grandes células extranodal primário do osso em um paciente de seis anos de idade, do sexo masculino, com manifestaçãoclínica e radiológica inicial em localização não-comum (tálus).

A case of a 6-year-old male primary B-cell non-Hodgkin's lymphoma with lytic bone lesion in an unusual location was demonstrated by clinical and radiologic examination.

Valor pronóstico de los niveles séricos del receptor a soluble de interleuquina-2 en pacientes con linfoma No Hodgkin / Prognostic value of serum soluble interleukin-2 a receptor levels in Non Hodgkins lymphoma patients

El receptor a soluble de la interleuquina 2 (sRaIL-2) ha sido considerado un marcador pronóstico en pacientes con linfoma No Hodgkin (LNH). El objetivo del trabajo consistió en determinar la significancia de los niveles del sRaIL-2 en pacientes con LNH, su correlación con factores clínico patológicos, biológicos y su valor pronóstico. Se procesaron muestras de suero de 43 nuevos casos de pacientes con LNH, pretratamiento, edades comprendidas entre 25-84 años, mediana 55 años, 33 (77%) LNH agresivos y 10 (23%) LNH indolentes. Según el Índice Pronóstico Internacional (IPI), 37 (86%) de bajo riesgo y 6 (14%) de alto riesgo, siendo el tiempo promedio de seguimiento de la enfermedad 30 meses (rango 3-48 meses). Se recolectaron 20 muestras de suero de controles sanos, donantes de banco de sangre, para determinar el valor de referencia. Las muestras de pacientes y controles se congelaron a -70ºC hasta su procesamiento. El sRaIL-2 se determinó por un inmunoensayo enzimático adsorbente (ELISA), sensibilidad 10 pg/ml y se consideraron elevados los niveles >2000 pg/ml. Los marcadores biológicos: Beta 2 microglobulina (B2M), Proteína C Reactiva (PCR), Velocidad de sedimentación globular (VSG), Albúmina sérica, Lactato deshidrogenasa (LDH), Aspartato amino transferasa (AST), Alanino amino transferasa (ALT) y Fosfatasa alcalina (ALP), se determinaron por métodos de inmunoensayo enzimático sobre micropartículas (MEIA), aglutinación directa, Wintrobe, colorimétrico y cinético, respectivamente. Los niveles séricos del sRaIL-2 en pacientes con LNH fueron significativamente superiores al del grupo control (p <0,001) y 26 (60%) de los pacientes expresaron niveles elevados del sRaIL-2 (p< 0,001). Se detectaron niveles del sRaIL-2 significativamente incrementados asociados a pacientes con estadio clínico (EC) III-IV (p= 0,02), síntomas B (p= 0,04), valores elevados de B2M (p= 0,01) y PCR (p= 0,05)...

Soluble Interleukin–2 a receptor (sIL-2aR) has been considered as a prognostic marker in Non-Hodgkin’s lymphoma (NHL) patients. The aim of this study was to assess the significance of sIL-2aR levels in the pre-treatment serum of NHL patients, as well as its correlation with the clinical-pathological and biological factors and its prognostic value. Forthy-three serum samples from newly diagnosed NHL patients were processed before starting the treatment. The median age was 55 years old (range 25-84 years), 33 cases (77%) were classified as aggressive lymphomas and 10 cases (23%) as indolent lymphomas. According to the International Prognostic Index (IPI), 37 cases (87%) were classified as high risk group and 6 cases (14%) as low risk group. The average time of follow–ups was 30 months (range 3-48 months). 20 healthy control samples from blood bank donors were collected for establishing the reference value. All patients and control samples were kept at -70ºC until processed. The sIL-2aR levels were determined by applying an enzyme-linked immunosorbent assay (ELISA), sensitivity 10 pg/ml. Values were considered elevated when they exceeded 2000 pg/ml. The biological markers: Beta 2 microglobulin (B2M), C-Reactive Protein (CRP), erythrocyte sedimentation rate (ESR), serum albumin, serum lactate dehydrogenase (LDH), aspartate amino transaminase (AST), alanine amino transaminase (ALT), alkaline phosphatase (ALP), were determined by the methods of microparticle enzyme immunoassay (MEIA), direct agglutination, Wintrobe, colorimetric and kinetic respectively. The sIL-2aR serum levels in the NHL patients were significantly higher than the control group (p< 0.001) and 26 (60%) of the patients expressed values higher than 2000 pg/ml (p<0.001). Significantly elevated serum levels of sIL-2aR were associated with clinical stage (CS) III-IV (p= 0.02), B symptoms (p= 0.04), elevated levels of B2M (p=0.01) and CRP (p=0.05)...

Tratamiento de pacientes con linfomas no Hodgkin asociados a SIDA: experiencia del Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán / Treatment of non-Hodgkin's lymphoma associated with acquired immnodeficiency syndrome (AIDS) at the Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán

Antecedentes: La supervivencia de los pacientes con linfomas no Hodgkin asociados al Síndrome de Inmunodeficiencia Adquirida (SIDA) ha mejorado con el uso de antirretrovirales y de quimioterapia menos tóxica. Material y métodos: El objetivo del estudio fue mostrar los resultados del tratamiento de los pacientes con linfomas no Hodgkin y SIDA. Se estudiaron nueve pacientes de manera retrospectiva. Se analizó la supervivencia global y libre de enfermedad mediante curvas de Kaplan-Meier; además de sus características generales. Resultados: El tratamiento recibido fue a base de etopósido, prednisona, vincristina, doxorrubicina y ciclofosfamida (DA-EPOCH). Los pacientes tuvieron supervivencia global de 18 meses, una supervivencia libre de enfermedad de 13 meses, una mediana de seguimiento de 16 meses con respuestas completas en ocho de nueve pacientes. Conclusiones: Se observó una adecuada repuesta a tratamiento en este grupo de enfermos reflejada en una mayor supervivencia global.

BACKGROUND: Survival in patients with acquired immunodeficiency syndrome (AIDS) related non-Hodgkin's Lymphoma has improved with the use of High Active Antiretroviral Therapy (HAART) and less toxic chemotherapy. MATERIAL AND METHODS: Clinical characteristics and outcome among patients treated for AIDS related non-Hodgkin's Lymphoma are described. Nine patients were studied retrospectively. Overall survival (OS) and Free Disease Survival (FDS) using a Kaplan-Meier model were analyzed. RESULTS: Patients received (DA-EPOCH) etoposide, prednisone, vincristine, doxorubicin and cyclophosphamide. The overall Survival was 18 months and 13 month Free Disease Survival with a median follow-up of 16 months showing full response in 8/9 patients was observed. CONCLUSIONS: A very satisfactory treatment response in this group of patients expressed as an increased Overall Survival was noted.

Linfoma no Hodgkin de células B con extensión secundaria a glándula mamaria: Evaluación por imagen / Secondary non-Hodgkin's B-cell lymphoma involving the breast: radiologic imaging

Los linfomas secundarios con afectación de la glándula mamaria, aunque raros, representan el grupo más grande de tumores metastásicos de la mama. El linfoma no Hodgkin primario de la mama es también raro, representando del 1.7%-2.2% de los casos de linfoma no Hodgkin extranodal y del 0.38-0.7% de todos los linfomas no Hodgkin. Aproximadamente 300 casos han sido reportados en la literatura. El aspecto mamográfico ha sido descrito como áreas redondeadas u ovales con densidad aumentada. Las masas mamarias pueden aparecer homogéneas o heterogéneas. Las lesiones pueden ser muy bien definidas y ser confundidas con procesos benignos, esto último más probable en pacientes menores de 35 años de edad. Los hallazgos ultrasonográficos son descritos como masas de márgenes bien definidos con ecos de intensidad baja a moderada. Las paredes posteriores de la masa suelen estar bien definidas, probablemente con ligero reforzamiento acústico pero sin sombra posterior asociada. En resumen, el aspecto mamográfico del linfoma mamario no es específico, pero el diagnóstico puede ser excluido si hay presencia de calcificaciones o reacción desmoplásica. El tejido linfoide prominente en un paciente con masas mamarias debería despertar la sospecha de linfoma mamario. El aspecto más crítico en el estudio de una masa de la mama es la biopsia del tejido, puesto que una lesión linfomatosa tiene radiográficamente patrones indeterminados. Presentamos el caso de una mujer de 79 años con dolor abdominal y una masa palpable en la mama derecha; realizamos un breve resumen de las características clínicas y principales hallazgos por imagen del linfoma no Hodgkin con afección a la glándula mamaria (radiografía de tórax, mastografía, ultrasonido mamario, y tomografía de tórax).

Secondary lymphomas involving the breast, although uncommon, represent the largest group of metastasic tumors to the breast. Primary non-Hodgkin lymphomas (NHLs) of the breast are also rare, accounting for 1.7% to 2.2% of extranodal NHL cases and 0.38 to 0.7% of all NHLs. Approximately 300 cases have been reported in the Medical literature. Mammographic appearances are described as round or oval areas of opacity. The breast mass may appear homogeneous or inhomogeneous. The lesion may be very well defined and may be mistaken for a benign process, most notable in patients younger than 35 years of age. Ultrasonographic appearance is described as a sharply defined mass with low or medium echoes. The posterior aspect of the mass is well defined (possibly with slight acoustic enhancement but with no associated posterior shadowing). In summary, the mammographic appearance of the breast lymphoma is nonspecific, but the diagnosis can possibly be excluded if calcifications or a desmoplastic reaction are present. Prominent lymph vessels in a patient with a breast mass should raise the suspicion of breast lymphoma. The most critical aspect in the workup of a breast mass is the tissue biopsy, since radiographically lymphomatous lesions are indeterminate. We present the case of a 79-year-old woman with abdominal pain and a palpable breast mass; we also make a brief summary of the clinical features and main imaging findings of NHL (plain radiograph, mammography, breast ultrasound and thorax tomography).

A Case of Hodgkin`s Lymphoma with Bone Marrow Involvement Showing Severe Osteosclerosis / 대한진단검사의학회지

Bone marrow involvement in Hodgkin`s lymphoma is not rare at initial diagnosis and is associated with a poor prognosis. Atypical findings such as granuloma, eosinophilia or osteosclerosis may lead to diagnostic confusion, in which case immunohistochemistry may be helpful. We report a case of 25-year old male patient, who presented with back pain, subsequently had a mediastinal mass and was finally diagnosed as having stage IV Hodgkin`s lymphoma. Bone marrow involvement with severe osteosclerosis was found on bone marrow biopsy specimens. He received systemic chemotherapy and was discharged with improved clinical symptoms.

Presencia del virus de Epstein-Barr en casos colombianos de linfoma de Hodgkin y su relación con la respuesta al tratamiento / Epstein-Barr virus presence in Colombian Hodgkin lymphoma cases and its relation to treatment response

En el desarrollo y patogénesis del linfoma de Hodgkin se ha propuesto al virus de Epstein-Barr como posible factor etiológico debido a la detección de ADN viral en las células de Reed-Sternberg en un subgrupo de tumores y a los altos niveles de expresión de la proteína latente de membrana 1 (LMP-1). El objetivo de este estudio fue determinar la presencia de virus de Epstein-Barr en 67 ganglios linfáticos de pacientes con diagnóstico confirmado de linfoma de Hodgkin mediante hibridación in situ para la detección de transcritos de ARN del virus de Epstein-Barr e inmunohistoquímica para la detección de la proteína oncogénica LMP-1. La presencia del virus se relacionó con el subtipo histológico, la respuesta al tratamiento de los pacientes y el fenotipo del infiltrado linfocitario. En el 67% de los casos se detectaron transcritos de virus de Epstein-Barr, la proteína LMP-1 se detectó en 56,7% de los casos en la célula tumoral de Reed-Sternberg. La presencia del virus en cada tipo histológico fue de 69,81% en esclerosis nodular, 85,71% en celularidad mixta y 40% en clásico. El virus de Epstein-Barr se detectó con mayor frecuencia en niños (84,2%) en comparación con los adultos (60,4%) y los pacientes positivos para el virus mostraron mejor respuesta al tratamiento, reflejada en una menor tendencia a presentar recaídas. El análisis del infiltrado mostró un predominio de linfocitos T CD4 y presencia de linfocitos T CD8, con mayor expresión de ambas subpoblaciones en casos positivos para virus de Epstein-Barr. Los resultados muestran un alto porcentaje de infección por virus de Epstein-Barr con una probable implicación significativa en la respuesta al tratamiento, lo que sugiere que la detección de virus de Epstein-Barr se podría usar como marcador de pronóstico en este tipo de linfoma.

The role of Epstein-Barr virus as etiologic agent in Hodgkin lymphoma (HL) development has been supported by the detection of viral DNA in the Reed-Sternberg cell in a subset of HL, and the high levels of latent membrane protein 1 expression in these tumors. To gain further evidence of this relationship, lymph nodes from 67 patients with HL were analyzed for the presence of Epstein-Barr virus using EBERs in situ hybridization and LMP-1 immunohistochemistry. Virus presence was related to histological subtype, patients´ treatment response and tumor infiltrating lymphocytes phenotype. EBERs transcripts were found in 67% of the cases and LMP-1 in the Reed-Sternberg tumor cells at a 56.7% rate. The prevalence, as determined by histological subtype, was 69.81% for nodular sclerosing, 85.71% for mixed cellularity and 40% for lymphocyte-rich. Epstein-Barr virus presence was more frequent in children (84.2%) in comparison with adults (60.4%). Positive patients presented higher failure-free survival rates than Epstein-Barr virus negative patients. CD4 positive infiltrating T cells were present in a higher proportion in relation to CD8 positive T infiltrating cells, the mean percentages for both subsets were higher in Epstein-Barr virus positive cases. A high percentage of Epstein-Barr virus was present in HL with a probable association with treatment response. This suggests an application of Epstein-Barr virus detection to use as a prognosis marker in treatment response for HL cases.