Linfoma de Burkitt en un escolar con infección perinatal por VIH lentamente progresiva / Burkitt's lymphoma in a school boy with slow progression perinatally acquired HIV

Resumen Las manifestaciones clínicas en los niños con infección por el virus de la inmunodeficiencia humana (VIH) de transmisión perinatal, pueden ser de inicio precoz o tardío. El linfoma asociado a VIH es una manifestación tardía que se asocia a estadios avanzados de inmunosupresión. Se presenta el caso de un escolar de 9 años con diagnóstico de novo de infección por VIH que debutó con un linfoma de Burkitt. En niños, la frecuencia de esta asociación es de 1-2% con pocos casos reportados en la literatura médica.

Children with perinatal human immunodeficiency virus (HIV) infection can present early or late clinical disease. HIV-associated lymphoma is a later manifestation that is associated with advanced immunosuppression (acquired immunodeficiency syndrome -AIDS). This is a case of a 9-year-old boy with recent diagnosis of HIV with Burkitt's lymphoma as first clinical manifestation. In children, the frequency of this association is very low and there are few cases reported.

Linfomas plasmoblásticos del tracto gastrointestinal en pacientes con sida / Plasmablastic lymphoma of the gastrointestinal tract in AIDS patients

Los pacientes con infección por el virus de inmunodeficiencia humana (HIV) tienen 200 veces más riesgo de desarrollar un linfoma no Hodgkin (LNH) con respecto a la población general. El linfoma plasmoblástico (LP) representa menos del 3% de todos los LNH asociados con el HIV. El objetivo de este estudio es informar las características clínico-patológicas de 5 pacientes con enfermedad HIV/sida y LP del tracto gastrointestinal. Se revisaron de forma retrospectiva los casos de LP del tracto gastrointestinal diagnosticados en el Instituto Nacional de Cancerología de la Ciudad de México en el periodo comprendido entre los años 2000 al 2009. Se analizaron las características clínico-patológicas y se realizaron cortes de bloques de tejidos embebidos en parafina para reacciones de inmunohistoquímica. La presencia del virus de Epstein Barr (VEB) se examinó por reacción en cadena de la polimerasa (PCR) in situ. De los cinco pacientes, cuatro fueron hombres y una mujer, con una mediana de edad de 29 años. Tres tumores se localizaron en la región anorrectal, uno en colon ascendente y el restante en el estómago. Histológicamente, todos los tumores se caracterizaron por una proliferación difusa de células grandes de aspecto plasmoblástico. Las células neoplásicas fueron CD 138/MUM-1 positivas y CD 20 / PAX-5 negativas. En cuatro pacientes se detectó el genoma del VEB en las células neoplásicas mediante PCR in situ. La mediana de seguimiento fue 18 meses; tres pacientes estaban vivos con enfermedad y dos sobreviven sin evidencias de la neoplasia. El diagnóstico precoz de LP como una entidad clínico-patológica es importante para establecer el tratamiento correcto y mejorar el pronóstico de estos pacientes.

The risk of developing non-Hodgkin lymphoma (NHL) is 200 times higher in HIV-positive patients than otherwise healthy persons. Plasmablastic lymphoma (PL) represents < 3% of all NHL associated with HIV infection. The aim of this study was to review the clinical-pathologic features of PL of the gastrointestinal tract in 5 patients with HIV/aids disease. We performed a retrospective study of PL of the gastrointestinal tract diagnosed at the National Institute of Cancer at Mexico City, from 2000 to 2009. Clinical and pathological information was obtained and immunohistochemical studies were performed in paraffin-embedded tissue sections. The presence of Epstein-Barr Virus (EBV) was examined by in situ polymerase chain reaction (PCR). Four male and 1 female were included with a median of age of 29 years. Three tumors involved the ano-rectal area, one tumor the ascendant colon and one tumor the stomach. All tumors were histologically characterized by a monotonous proliferation of large lymphoid cell with plasmablastic features. Tumor cells were CD 138 / MUM-1positive and CD 20 / PAX-5 negative in all cases. EVB genome was detected by in situ PCR in 4 cases. The median of follow-up was 18 months, and revealed that three patients are alive with neoplasm disease and two patients are still alive with no evidence of the neoplasm. Recognition of this entity by pathologists and clinicians is important in order to establish the correct diagnosis and the early treatment of these patients.

Linfomas asociados con la infección por el virus de la inmunodeficiencia humana: subtipos histológicos y asociación con los virus de Epstein Barr y Herpes-8 / AIDS related lymphomas: Histopathological subtypes and association with Epstein Barr virus and Human Herpes virus type-8

Los linfomas no Hodgkin (LNH) constituyen la segunda neoplasia definitoria de Sida más frecuente. En el presente trabajo se evaluaron 48 casos de linfomas asociados con la enfermedad debida al virus de la inmunodeficiencia humana (HIV) diagnosticados en la División Histopatología del Instituto de Investigaciones Hematológicas de la Academia Nacional de Medicina. Se incluyeron en la investigación 5 mujeres y 43 hombres con una mediana de edad al momento del diagnóstico de la neoplasia de 37 años. La evaluación morfológica se realizó en cortes coloreados con hematoxilina-eosina, estudio inmunohistoquímico para la detección del virus de Epstein Barr (VEB) en 48/48 casos, y mediante sonda oligonucleotídica biotinilada para la detección del ADN del Herpes virus humano tipo-8 (HHV-8) en 14/14 linfomas plasmoblásticos (LP). Todos fueron linfomas de fenotipo B, con un curso clínico agresivo y enfermedad neoplásica avanzada al momento del diagnóstico. Se pudo demostrar la fuerte asociación del VEB con los linfomas asociados al sida, con frecuencias que variaron según el subtipo histológico: 16/21 (76%) para los linfomas difusos de grandes células; 1/3 casos (33%) de linfomas de Burkitt y 3/4 (75%) en los linfomas primarios del sistema nervioso central. Globalmente, el genoma del VEB se detectó en 20/28 (71%) de las muestras de biopsias de LNH de esta serie. La detección del HHV-8 resultó negativa en los 14 LP. Los linfomas de Hodgkin fueron más frecuentes en varones,18/20 (90%), con un curso clínico agresivo y franco predominio de los subtipos histológicos de peor pronóstico (90% de casos). En estas neoplasias también se comprobó una frecuente asociación patogénica con el VEB (90% de casos).

Non-Hodgkin lymphomas (NHL) of the B-cell type are the second most common neoplasm among patients with human immunodeficiency virus (HIV) infection and AIDS. Here, we evaluated 48 cases of AIDS-related lymphomas (ARL) diagnosed at the Histopathological Division of the Instituto de Investigaciones Hematológicas of the National Academy of Medicine. Five were females and 43 were males with a median of age of 37 years at the time of the diagnosis. Micrometer sections were prepared and stained with hematoxilin-eosin; immunohistochemical examination for the presence of Epstein-Barr virus (EBV) was carried out in 48/48 cases. Additionally, biotinilated oligonucleotides were used to determine the presence of DNA of the Human Herpes virus type-8 (HHV-8) in 14/14 biopsy smears corresponding to plasmablastic lymphomas (PL). All were fenotype B cell lymphomas with an aggressive course and advanced neoplasm disease at the time of diagnosis. Virological findings showed the strong association between EBV and AIDS-related NHL. According to the histopathological subtype, the EBV genome was detected in 16/21 (76%) diffuse large B cell lymphomas, 1/3 Burkitt lymphoma and 3/4 (75%) of primary central nervous system lymphomas. Globally, EBV genome was detected in 20/28 NHL of this series. Detection of HHV-8 was negative in all cases of PL. Hodgkin lymphoma were more frequent in males 18/20 (90%), with an aggressive clinical course and a significant predominance of the subtypes associated with worse prognosis (90% of cases). We detected a significant association between EBV and HL (90% of cases). We consider that all cases of AIDS related lymphomas should be assessed for the presence of EBV because its presence may play a role in the prognosis.

Burkitt's lymphoma of the duodenum in a patient with AIDS

Non-Hodgkin's lymphoma of B-cell type is the second most common neoplasm after Kaposi's sarcoma, among patients with human immunodeficiency virus infection. Most non-Hodgkin's lymphoma cases that are associated with acquired immunodeficiency syndrome involve extranodal sites, especially the digestive tract and the central nervous system. We report a case of primary lymphoma of the duodenum in a patient with AIDS. Upper gastrointestinal endoscopy revealed pseudopolypoid masses found in the second portion of the duodenum. A complete diagnostic study including histological, immunohistochemical and virological analyses showed high-grade B-cell Burkitt's lymphoma. The Epstein-Barr virus genome was detected in biopsies by immunohistochemical and in situ hybridization.

O linfoma não-Hodgkin de células B é a segunda neoplasia mais comum em pacientes com infecção pelo vírus da imunodeficiência humana depois do sarcoma de Kaposi. A maioria dos casos de linfoma não-Hodgkin associados com a síndrome da imunodeficiência adquirida envolve locais extraganglionares, especialmente o trato digestivo e o sistema nervoso central. Nós relatamos um caso de linfoma primário do duodeno em um paciente com AIDS. Uma endoscopia digestiva alta mostrou massas pseudopolipóides encontradas na segunda porção do duodeno. Um estudo diagnóstico completo incluindo exames histológicos, imunohistoquímicos e virológicos mostrou um linfoma de células B tipo Burkitt. Detectou-se genoma do vírus Epstein-Barr em biópsias por hibridização in situ e imuno-histoquímica.

Primary lymphoma of the central nervous system: a clinical-pathological and immunohistochemical study of ten autopsy cases / Linfoma primário do sistema nervoso central: estudo clínico-patológico e imuno-histoquímico de dez casos de necropsia

CONTEXT: Primary central nervous system lymphomas (PCNSL) are a rare subgroup of lymphomas generally associated with HIV and EBV. OBJECTIVE: To study ten autopsy cases of PCNSL, to describe the neuropathological findings, to characterize the phenotype of the neoplastic cells, to detect EBV in the lesion and to compare the findings with the clinical and laboratory data of the patients. METHOD: The clinical, histological and immunohistochemical data of ten cases of PCNSL, eight cases from patients with AIDS, identified among 265 autopsies of these patients were analyzed. RESULTS: Seven patients were males and the mean age was 40.9 years. The most frequent symptomatology was focal neurologic deficit (70 percent). Six patients presented with only one lesion. Histologically, densely cellular and polymorphous neoplasms with angiocentrism were observed, in 90 percent of cases. An association with other diseases was observed in four cases. Most patients had diffuse large B cell non-HodgkinÆs lymphoma. EBV was detected by immunohistochemistry in only one case. The lack of detection of the virus might have been due to the long time of fixation of the brain which might have inactivate epitopes therefore compromising the testing. CONCLUSION: In the present series, PCNSL presented with focal symptoms, with unifocal or multifocal lesions, with a predominant B-cell CD20 positive phenotype, rarely associated with EBV.

CONTEXTO: Linfoma primário do sistema nervoso central (LP-SNC) é raro subgrupo de linfomas relacionado à AIDS, geralmente associado EBV. OBJETIVO: Identificar os achados clínico-patológicos dos pacientes com LP-SNC. MÉTODO: Foram analisados dados clínicos, histológicos e imuno-histoquímicos de dez necrópsias de LP-SNC, oito deles de pacientes com AIDS, identificados entre 265 autopsias destes. RESULTADOS: Sete pacientes foram masculinos e a idade média foi 40,9 anos. A sintomatologia neurológica mais freqüente era focal (70 por cento). Seis exibiram lesão única. Histologicamente, eram neoplasias densamente celulares e polimorfas, com angiocentrismo em 90 por cento dos casos. Em quatro casos, houve associação com outras afecções. A maioria dos casos foi de linfoma não-Hodgkin difuso de grandes células B. A pesquisa para EBV foi positiva em um caso. CONCLUSÃO: Predominaram os LP-SNC associados à AIDS, com sintomatologia focal, lesão em massa ou multifocal, com predominância de células B CD-20.