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.

Coexistencia de linfoma plasmablástico, sarcoma de Kaposi y enfermedad de Castleman en un paciente con infección por virus de inmunodeficiencia humana / Coexistence of plasmablastic lymphoma, Kaposi sarcoma and Castleman disease in a patient with HIV infection

El linfoma plasmablástico (LP) es un linfoma de células B poco común que está fuertemente asociado con la infección por el virus de inmunodeficiencia humana (VIH), y muestra una afinidad característica de presentación extra-ganglionar en la cavidad oral. Informamos el caso de un LP afectando el estómago en un paciente masculino de 36 años de edad con infección por VIH, asociado con sarcoma de Kaposi (SK) en áreas adyacentes al linfoma. Tenía el antecedente de enfermedad de Castleman y SK en una biopsia de ganglio linfático.

Plasmablastic lymphoma (PL) is an uncommon B-cell lymphoma that is strongly associated with human immunodeficiency virus (HIV) infection, and displays distinctive affinity for extranodal presentation in the oral cavity. We report the case of a PL involving the stomach in a 36 year-old man HIV+ patient, associated with Kaposi sarcoma (KS) in sections adjacent to lymphoma. He had a positive history of Castleman disease and KS in a lymphoid node biopsy.

Morphological spectrum of AIDS-related plasmablastic lymphomas.

We have had a recent spurt in cases of AIDS-related lymphoma (ARL) at our centre. Most of these cases are aggressive mature B cell lymphomas, mainly plasmablastic lymphoma (PBL) and diffuse large B-cell lymphoma (DLBCL). Most of the PBL are extranodal in location and are mucosa-based. We reviewed the morphological features of 34 cases of PBL. Diagnosis was based on morphology, immunohistochemistry, proliferation index, HIV positive status and its preference to extranodal sites (mostly mucosa based). We classified PBL into three morphological subtypes (immunoblastic - 25, Burkitt's - 7, plasmacytic - 2). Tumor cells expressed as leucocyte common antigen (LCA) in 60%, CD138 in 100%, EMA in 45% and light chain restriction in 86% cases. CD20 was negative in all cases. Pathologists need to be aware of PBL and its various morphological subtypes as the identification of this entity from its close differentials carries major therapeutic implications.

Peripheral T-cell lymphomas-clinicopathologic and immunophenotypic analysis of 25 cases

The clinicopathologic and immunophenotypic findings of 25 cases of peripheral T-cell lymphoma in Korea were analysed. Seventeen cases (68%) of the 25 T-cell lymphomas presented in the extranodal sites including the nasal mucosa, tonsil, oral cavity, skin and rarely bone, mediastinum and breast. Immunologic studies showed that 12 cases (48%) of the lymphomas were of T-helper phenotype, 5 cases (20%) were of cytotoxic/suppressor phenotype, 1 case (4%) expressed both helper and cytotoxic/suppressor markers, and 7 cases (28%) lacked detectable markers for subsets. Histologically, fourteen cases (56%) showed histologic features suggestive of peripheral T-cell lymphoma. The more frequently seen histologic types by Working Formulation (WF) included large cell type and immunoblastic type. Classification by WF was straightforward in most cases of large cell, immunoblastic type. However, with some cases of small cell, large cell and mixed types, there were problems fitting the morphology seen into the WF category. We hope that the establishment of a world wide immunologic and clinicopathologic classification for peripheral T-cell lymphoma will be made in the near future.