Linfoma primario difuso de células B grandes de mucosa gingival, en paciente VIH y EBV negativos / Primary gingival diffuse large b-cell lymphoma in HIV/EBR negative patient abstract

El linfoma difuso de células B grandes (LDCBG) es el linfoma más frecuente. La presentación clínica puede ser nodal o extranodal y sus síntomas dependen de la localización tumoral; en la mayoría de los casos están asociados a algún tipo de inmunodeficiencia. Referiremos un caso de LDCBG de presentación atípica en una localización muy infrecuente. Es importante tener en cuenta estas situaciones, ya que pueden simular otros procesos patológicos, retrasando así su correcto diagnóstico y por lo tanto un adecuado tratamiento. (AU)

Diffuse large cell lymphoma B (LDCBG) is the most common type of lymphoma. It´s clinical presentation can be nodal or extranodal and it's symptoms depend where the tumor is located and whether is associated or not with an immunodeficiency disease. We present an atypical presentation of a LDCBG in a very unusual location. It´s important to consider these kind of appearance, as they can mimic other oral pathological processes, delaying their correct diagnosis and therefore an appropriate treatment. (AU)

VIH y Linfoma, una reflexión necesaria: reposte de serie de casos / HIV and LIMPHOMA, a necessary reflection: Case Series Report

La relación entre el deterioro de la función inmunológica y el desarrollo de linfoma es bien conocido en los pacientes con algún grado de inmunosupresión, en los que se observan linfomas con mayor frecuencia que en la población en general, por lo tanto no es sorprendente la asociación entre el virus de inmunodeficiencia humana (VIH) y el desarrollo de este tipo de neoplasia, con - siderándose como definitoria de enferme- dad avanzada. Los linfomas no Hodgkin (LNH) constituyen la segunda neoplasia más frecuente asociada a la infección por VIH, identificándose dos formas anatomo- clínicas principales: Los LNH Sistémicos y los Linfomas primarios del sistema nervioso central (LPSNC), estos últimos se han reducido considerablemente desde la intro - ducción del tratamiento antirretroviral de gran actividad (TARGA). Aunque no se considera definitorio de enfer - medad avanzada, otras neoplasias relacio - nadas con VIH son Enfermedad de Hodgkin y leucemias. Se presenta a continuación una serie de 4 casos clínicos de pacientes con VIH, atendidos en el Centro de Atención integral pediátrico/ Hospital Nacional Doctor Mario Catarino Rivas (HNMCR), en el año 2013, donde se puede observar la variedad de presentación de los linfomas y su relación con el estado inmunológico y virológico...(AU)

Experiencia de linfomas cutáneos entre enero de 1995 y abril de 2008 en el Instituto Nacional de Cancerología / Experience with cutaneous lymphomas between January 1995 and April 2008 at the National Cancer Institute

Objetivos: Describir los diferentes linfomas cutáneos conforme a la clasificación WHO- EORTC, observados en el Instituto Nacional de Cancerología (INC) entre el 1° de enero de 1995 y abril de 2008. Métodos: Estudio retrospectivo, descriptivo, donde se incluyó a los pacientes con diagnóstico de linfoma cutáneo en el INC desde enero de 1995 hasta abril de 2008. La ubicación de las historias clínicas se realizó utilizando la base de datos del Departamento de Patología. El análisis estadístico se realizó mediante el programa Epi info 2008. Resultados: Se revisaron 252 historias y se incluyó a 160 pacientes en el estudio: 139 linfomas T (87%) y 21 linfomas B (13%). El linfoma más común fue la micosis fungoide (63% de los casos). Dentro de las variantes descritas de micosis fungoide (MF) llamó la atención la frecuencia de MF hipopigmentadas (13%). El grupo de enfermedades linfoproliferativas CD30+ fue el segundo en frecuencia dentro de los linfomas T. En el grupo de linfomas B los linfomas indolentes de excelente pronóstico se presentaron en un 5% de los casos. Conclusiones: Los linfomas cutáneos primarios son tumores raros. Predominan los linfomas de células T sobre los B. La mayoría de los casos son linfomas de bajo grado, y deben tratarse con terapias dirigidas a la piel. Dentro de cada categoría existen linfomas que van a progresar y comprometer órganos internos.

Objectives: To describe different cutaneous lymphomas in accordance with WHO-EORTC classification under observation at the National Cancer Institute (NCI) between January 1, 1995 and April, 2008. Methods: A descriptive, retrospective study was carried out which included patients diagnosed with cutaneous lymphoma at the NCI from January 1995 until April 2008. Clinical cases were taken from Pathology Department data base. Statistical analysis was performed with Epi 2008 info program. Results: A total of 252 case histories were reviewed, and 160 patients were included in the study: 139 T-cell lymphomas (87%) and 21 B-cell lymphomas (13%). The most common (63% of cases) was mycosis fungoides (MF). Among the variations of MF described, the frequency of hypopigmented MF (13%) stood out. The lymphoproliferative CD30+ disease group was the second most frequent among T-cell lymphomas. In the B-cell lymphoma group, the indolent lymphomas with excellent prognosis made up 5% of cases. Conclusions: Primary cutaneous lymphomas are rare tumors. T-cell lymphomas predominate over B-cell. Most cases are low grade lymphomas and should be treated with skin therapies. Within each category, lymphomas exist that will progress and compromise internal organs.

Malignant lymphomas in Pakistan according to the WHO classification of lymphoid neoplasms.

OBJECTIVE: To determine the spectrum of malignant lymphomas in our set up, according to the WHO classification. METHODS: All the cases diagnosed as malignant lymphoma, during the year 2005, were retrieved from the institution based tumour registry record and classified according to WHO criteria depending on the immunohistochemical results of a panel of lymphoma markers. RESULTS: The male to female ratio was 2.5:1 for almost all types of malignant lymphomas. The age range was 3 to 80 years. The frequency of Hodgkin's lymphoma, Burkitt's lymphoma and lymphoblastic lymphoma were higher amongst the children, whereas follicular lymphomas, mantle cell lymphoma and CLL/SLL were more frequently reported in 5th, 6th and 7th decades. Of the total cases 62% were nodal and 38% extranodal (majority in the GI tract). Non Hodgkin's lymphoma was more (73%) frequent than Hodgkin's disease. Mixed cellularity and nodular sclerosis were the main histological variants of Hodgkin's disease. CONCLUSIONS: Immunohistochemistry is not very frequently used in our set up and also at very few other centres. Therefore, its application should be encouraged to raise the quality of data on lymphoid neoplasms and contribute to their control.

Linfoma no Hodgkin en pacientes con SIDA: una reflexión necesaria / Non-Hodking lymphoma in AIDS patients: a necessary reflection

El linfoma no Hodgkin constituye una de las 3 afecciones malignas marcadoras de SIDA reconocidas actualmente. Tanto en Cuba, como en el resto del mundo, en la última década se ha observado su incremento en pacientes infectados por el virus de la inmunodeficiencia humana (VIH). Por las razones anteriormente planteadas, se realizó una revisión bibliográfica actualizada del tema en revistas de alto impacto internacional, para describir los principales elementos de esta enfermedad (clasificación, epidemiología, evolución y tratamiento) y brindar, de esta forma, una guía para el trabajo de los profesionales de la atención primaria en el seguimiento de estos pacientes en la comunidad.

Renal involvement in non-Hodgkin's lymphoma: the Shaukat Khanum experience.

BACKGROUND: Primary lymphoma of genitourinary system is rare as these organs do not contain lymphoid tissue, however secondary involvement often occurs. The most commonly affected genitourinary organ is the kidney. METHODS: Medical records of 901 patients with documented NHL seen at Shaukat Khanum Memorial Cancer Hospital & Research Center during 1995-2003 were studied for the incidence, histopathological, clinical and radiological correlation of renal involvement in NHL. RESULTS: 19(2.1%) patients had renal involvement. Male to female ratio was 3.75:1. Histology was diffuse large cell lymphoma in 12(63%) patients. IPI was High, High intermediate and Low intermediate in 17(89.5%) patients. Radiologically, 5(26.5%) patients had the disease above the diaphragm, 2(10.5%) patients had disease below the diaphragm while 12(63%) had disease on both sides of the diaphragm. 11(58%) showed complete response, 1(5.5%) showed partial response while 7(36.8%) showed progressive disease. CONCLUSION: Majority of patients with renal involvement had low intermediate or higher IPI compatible with significant progression rate. The findings and disease behavior in our population is comparable to those quoted in English literature. Radiological tools have made it easier to monitor disease response and renal biopsy is seldom required.

Linfoma: epidemiología y clasificación actual. Trabajo de actualización / Lymphoma: epidemiology and current classification

La incidencia del linfoma no-Hodgkin (LNH) ha aumentado aproximadamente un 80 por ciento desde la década de 1970 y ahora es el quinto tipo de cáncer más común en los Estados Unidos. La incidencia de LNH es aproximadamente un 50 por ciento más alta en hombres que en mujeres y un 35 por ciento más alta en la raza blanca que en la raza negraq. Las tasas de incidencia de todos los subtipos de LNH ha aumentado, especialmente el subtipo de célula grande difusa y el inmunoblástico. El aumento de la incidencia de LNH es poco comprendido. Los factores de riesgo arrojan resultados contradictorios: algunos de estos factores son: la herencia, la distribución geográfica, el tabaco, el alcohol, las enfermedades auatoinmunes, el medio ambiente, la exposición ocupacional, las tareas domésticas. Futuros estudios epidemiológicos deberían incorporar la nueva clasificación de la Organización Mundial de la Salud, basada en estudios genotípicos y fenotípicos para mejorar el diagnóstico de LNH.

Linfoma No Hodgkin (LNH) asociado a SIDA en la cavidad bucal: reporte de caso clínico / AIDS-related Non-Hodgkin lymphoma in the oral cavity: report of a clinical case

El riesgo de Linfoma No Hodgkin (LNH) en las personas con infección por el Virus de Inmunodeficiencia Humana (VIH) es cien veces mayor respecto al resto de la población. La incidencia de estas neoplasias en hombres jóvenes en la década de los 80 aumentó veinte veces producto de la infección por el VIH. No obstante, en los últimos años, el Síndrome de Inmunodeficiencia Adquirida (SIDA) es factor de riesgo condicionante de una discreta proporción de los casos de LNH. Estos linfomas suelen presentarse en su mayoría de células B con histología de alto grado, principalmente del subtipo difuso de células grandes, con compromiso extraganglionar frecuente y principalmente en el sistema nervioso central (SNC). En general, el entorno clínico y la respuesta al tratamiento de los pacientes con linfoma relacionado con el SIDA son muy diferentes a los de los pacientes seronegativos con linfomas. El individuo con linfoma agresivo que presenta infección por VIH se presenta generalmente con enfermedad en etapa avanzada que suele ser extraganglionar. Su curso clínico es más agresivo, la enfermedad es más extensa y menos sensible a la quimioterapia. Presentamos un caso clínico de Linfoma No Hodgkin en cavidad bucal en un paciente con las características antes mencionadas que acudió al Centro de Atención a Pacientes con Enfermedades Infectoectocontagiosas "Elsa La Corte" - CAPEI de la Facultad de Odontología de la Universidad Central de Venezuela, referido por el Centro de Hematooncología de la misma universidad, del cual destacamos el manejo interdisciplinario médico-odontológico del mismo.

Non-Hodgkin's Lymphoma's risk (NHL) in the persons with infection for the Human of Immunodeficiency Virus (HIV) is hundred times major with regard to the rest of the population. The incidence of these neoplasms in young men, in the decade of the 80 increased twenty times product of the infection for the HIV. Nevertheless, in the last years, the Acquired Immunodeficiency Syndrome (AIDS) is a cause of a discreet proportion of NHL's cases. These lymphomas are in the habit of appearing in the main of cells B with histology of high degree, principally of the diffuse subtype of big cells, with extranodal frequent compromise and principally in the nervous central system (NCS). In general, the clinical environment and the response to the treatment of the patients with lymphoma related to the AIDS are very different from those of the HIV_negative patient with lymphomas. The individual with aggressive lymphoma that presents infection for HIV appears generally with disease in advanced stage that is in the habit of being extranodal. The clinical course is more aggressive, the disease is more extensive and less sensitive to the chemotherapy.We present Non-Hodgkin's Lymphoma's clinical case in oral cavity in a patient with the characteristics before mentioned that came to the Center of Attention to Patients with Infectious and Contagious Diseases "Dra. Elsa La Corte" of the Faculty of Odontolgy of the Central University of Venezuela, refered by Hematooncology's Center of the same university.

Immunohistochemical typing of non-Hodgkin's lymphoma-comparing working formulation and WHO classification.

The recent WHO classification of non-Hodgkin's lymphoma is based on the morphology and immunohistochemical expression of the lymphoma cells and to a lesser extent, on the molecular and cytogenetic findings. Fifty-three cases of non-Hodgkin's lymphoma were included in the study. Of these, seven cases were primary extra nodal lymphomas. Twenty two patients had peripheral blood and/or bone marrow involvement. A detailed morphological assessment was done and classified using the International working formulation. The two most common types encountered were diffuse large cell lymphoma and small lymphocytic lymphoma. Immunohistochemistry was done using labeled streptavidin-biotin peroxidase complex with CD3, CD20, CD15, CD30, CD 45 (leukocyte common antigen), Cyclin D1, EMA (epithelial membrane antigen). 38 cases (72%) showed B cell expression and 12 cases (22.5%) showed T cell expression. Three cases did not express either marker. B-cell diffuse large cell lymphoma (26%) was found to be the predominant B cell non-Hodgkin's lymphoma. The commonest T-cell lymphoma was T lymphoblastic lymphoma (67%) followed by peripheral T cell angioimmunoblastic lymphoma (25%). Immunohistochemistry is a useful and necessary diagnostic modality and helps subdivide prognostically different types of non-Hodgkin's lymphoma.

Gastrointestinal lymphoma in Thailand: a clinicopathologic analysis of 120 cases at Siriraj Hospital according to WHO classification.

Clinicopathologic information of gastrointestinal (GI) lymphoma in Southeast Asia is lacking. A retrospective analysis of 120 cases of GI lymphoma in Thailand diagnosed at Siriraj Hospital based on WHO classification was performed. All were non-Hodgkin lymphoma (NHL). The peak age was in the sixth and seventh decades; a slight male preponderance was observed. Sites of involvement included stomach (49.2%), intestine (46.7%), and multiple sites (4.2%). There were 104 cases of primary GI lymphoma (86.7%) and 16 cases of secondary GI lymphoma (13.3%). Presenting GI symptoms were more common in the former; while superficial lymphadenopathy and fever were more common in the latter. Mass lesions were observed in both groups (72.1% vs 56.3%). Localized and advanced diseases were found in 68.3% and 31.7% of primary GI lymphomas, respectively. The most common type of lymphoma in both groups was diffuse large B-cell lymphoma. Lymphoepithelial lesions (LEL) were not significantly different between the two groups (58.2% vs 42.9%), but Helicobacterpylori infection was significantly associated with primary gastric lymphoma (p < 0.0001). The treatment of choice for localized primary GI lymphoma is controversial. Complete surgical resection may increase the chance of complete remission, but mortality and relapse rates might be higher than those observed with combination chemotherapy alone. GI lymphomas in Thailand are mostly primary B-cell NHL. LEL is not indicative of primary GI lymphoma, but H. pylori infection is closely associated with primary gastric lymphoma. A prospective study to determine the treatment of choice for localized GI lymphoma is needed.

Non-Hodgkin Lymphoma in Jordan. Types and patterns of 111 cases classified according to the WHO classification of hematological malignancies

Non-Hodgkin lymphoma [NHL] is one of the most frequent malignancies in Jordan. The aims of this study are: 1. To classify NHL cases in Jordan, using the new World Health Organization [WHO] classification system, 2. To identify the most common types of NHL in Jordan, and 3. To compare lymphoma types and patterns in Jordan with those in surrounding countries and the West. We studied all NHL cases, diagnosed during 1996 through to 1999 inclusive, at 2 major medical centers in Jordan, in order to identify their main types and patterns. One hundred and eleven cases of confirmed NHLs were reexamined and immunophenotyped in the year 2000, at the Department of Pathology, Jordan University of Science and Technology, Irbid, Jordan, using an immunohistochemical panel of antibodies, including CD3, CD15, CD20, CD30, CD43, CD45 and CD45RO. Confirmed NHL cases were reclassified according to the recently proposed WHO system of Hematological Malignancies. The median age of NHL cases was 44-years [range 2-85]. The vast majority of cases were of B-cell phenotype; only 14% of the cases were T-cell lymphomas. Most of the cases were of the aggressive intermediate to high-grade large cell type. Diffuse large B-cell lymphoma [DLBCL] comprised 53% of NHLs and 62% of B-cell NHLs. Indolent lymphomas were uncommon, comprising 14% of all NHL cases. Twenty-nine cases were seen among patients less than 20-years. Burkitt lymphoma represented the largest group [55%] of the childhood NHLs followed by diffuse large cell and lymphoblastic types. Indolent lymphomas are rare in Jordan and account for less than 15% of all NHLs. Aggressive lymphomas; on the other h and, account for the majority of NHLs in Jordan. Burkitt lymphoma affected children less than 10-years of age with a median of 4.5-years. These observations indicate that NHLs in Jordan have different type distribution and patterns from those seen in the West

Envolvimento secundário pulmonar por linfomas e leucemias / Secondary lung involvement by lymphomas and leukemias

Estudamos o envolvimento secundário pulmonar por leucemias em estudo retrospectivo de 12 anos de autópsias. Descrevemos o padrão de infiltração pulmonar em 77 linfomas e 50 leucemias. Os linfomas apresentaram maior freqüência do padrão peribrônquico/ perivascular de infiltração. O padrão intersticial foi mais freqüente nas leucemias. Mais de um padrão de infiltração estavam presentes por paciente. Linfomas de Hodgkin apresentaram maior freqüência do padrão nodular em relação aos linfomas B e T. Linfomas T apresentaram maior freqüência de infiltração intersticial do que os linfomas B / We have studied the secondary lung involvement by lymphomas and leukemias in a retrospective 12-year autopsy study. We have described histological infiltration patterns in lymphomas and leukemias. Lymphomas showed peribronchial/perivascular infiltration pattern. Leukemias had more frequently interstitial pattern. More than one infiltration pattern was present per patient. Hodgkin lymphomas had increased frequency of nodular infiltration pattern than B or T cell lymphomas. T cell lymphomas had more frequent interstitial pattern than B cell lymphomas. Pleural and intrapulmonary lymphnode involvement was more frequent in lymphomas than in leukemias...

Pax5 expression in Non-Hodgkin's Lymphomas and Acute Leukemias

The Pax5 gene encodes the B-cell-specific activator protein which is a key regulator in development and differentiation of B-cell. We studied the expression of Pax5 in hematologic malignancies to evaluate the diagnostic utility as a B cell marker. Materials included 70 B cell lymphomas, 26 T cell lymphomas, 53 acute leukemias, and 6 multiple myelomas (MMs). Representative areas from the paraffinembedded tissues were selected for tissue microarray, and the expressions of Pax5 was immunohistochemically evaluated. Pax5 was strongly expressed in most of the B cell lymphomas; 44 of 47 diffuse large B cell lymphomas (93.6%), 15 of 16 marginal zone B cell lymphomas (93.8%), all 3 mantle cell lymphomas, 2 follicular lymphomas, and 2 Burkitt's lymphomas (100%). However, Pax5 was expressed in only one of 26 T cell lymphomas. Among leukemias, it was expressed in 10 of the 14 B acute lymphocytic leukemias (ALLs) (72.4%), but also in 3 of the 6 T ALLs (50%), 13 of the 26 acute myelogenous leukemias (AMLs) (50%) and in all 3 ALL arising in chronic myelogenous leukemias and 4 mixed B ALL and AML. In MMs, Pax5 was negative in all cases. We concluded that Pax5 is very useful B cell marker in classification of lymphomas, but not of acute leukemias.

Linfomas näo Hodgkin: atualizaçäo e aspectos controversos / Non-Hodgkin's lymphomas: actualization and controversial aspects

Os autores apresentam uma breve revisäo sobre os linfomas näo Hodgkin, destacando aas principais características quanto à clínica, patologia, diagnóstico e terapêutica dos mesmos. Além desse aspecto, centram, principalmente, seus tópicos de discussäo nos aspectos relevantes sobre as diversas classificaçöes até entäo propostas aos linfomas e já descritas na literatura mundial.

Classificação dos linfomas não-Hodgkin: REAL e OMS / Classification of non-Hodgkin´s lymphomas: REAL and WHO

Apresento neste relato a nova classficação dos linfomas não-Hodkins derivada da proposição do grupo de patologistas de estudo de linfomase a correlação da mesma com a classificação adotada pela Organização Mundial de Saúde a partir de 1999.

The new classification of non-Hodgkin'slymphomas derived from the proposal of the International Lymphoma Study Group and the correlation between this and the classification adopted by the World Health Organization is related in this report.

Central nervous system lymphomas: a histologic and immunophenotypic analysis

OBJECTIVE: To observe the spectrum of non-Hodgkin's lymphomas involving the central nervous system including morphological subtypes and immunophenotypic status. SETTING: Retrospective analysis of eleven years [1986 to 1996] data from surgical pathology files of Department of Pathology. Forty-three cases of non-Hodgkin's lymphomas were diagnosed during the period of eleven years [from 1986 to 1996], all of which were diffuse types. A total of 1177 Central Nervous CNS biopsies were examined, out of which 937 cases were diagnosed as CNS neoplasms, the remaining were non-neoplastic in nature. Among 937 CNS neoplasms, 43 cases [4.6%] were reported as non-Hodgkin's lymphomas. As most of the cases were outside referrals, the primary or secondary nature of the lymphomatous process could not be assessed. Seventeen cases were intracranial, while 26 cases were spinal in location. Majority of the intracranial lymphomas were biopsied from the cerebrum [12 cases]. Male to female ratio was 1:2. The median age for intracranial lymphomas was 50 years and for spinal lymphomas 29 years. There were 16 cases [37%] of diffuse large cell lymphomas; 7 cases [16%] of diffuse mixed small and large cell lymphomas; 3 cases [7%] of diffuse large cell immunoblastic lymphomas; 2 cases [4.6%] of lymphoblastic lymphomas and diffuse small non-cleaved cell lymphomas and one case of small lymphocytic lymphoma and diffuse small cleaved cell lymphoma. One case of T cell rich B cell lymphoma was also diagnosed in the thoracic spine as primary extranodal lymphoma. Eight cases were unclassifiable and in 2 cases the features were suggestive of lymphoma. Immunophenotypic analysis was performed in 20 cases, however, in 2 cases the results were inconclusive. Fifteen cases [83%] showed immunoreactivity for B cell markers and 3 cases showed T cell phenotype out of which one case was lymphoblastic lymphoma. CNS lymphomas were uncommon tumors and comprised 4.6% of the total CNS neoplasms in our study. Moreover, these CNS lymphomas accounted for 2.2% of the total non-Hodgkin's lymphomas, including both nodal and extranodal. There was a higher incidence of location of these lymphomas within the spinal cord than brain. Most of the lymphomas were of intermediate or high grade [75%] according to the working formulation. Immunophenotypical status revealed B-cell phenotype in 84% of the lymphomas, in which it was tested [JPMA 50:141, 2000]

Pediatric solid cancer; a larkana experience

This prospective study was conducted to evaluate pattern and frequency of solid pediatric cancers of children in upper Sindh. Total number of cases was 214. Male to female ratio was 1.97:1 and mean age of patients was 8.7 years. Maximum number of patients were in 10-15 year age group [43.94%]. The five most frequent tumours were lymphoma [46.29%], soft tissue sarcoma [19.62%], retinoblastoma [11.68%], bone tumour [9.34%] and Wilm's tumour [7.0%]. CNS tumours were not represented in the study due to lack of neurosurgical facilities. Retinoblastoma and Wilm's tumour were more common in 0-4 year age group, whereas rest of the tumours were more frequent in 5-9 and 10-15 year age groups. Hodgkin's disease [62 cases] was more common than non-Hodgkin's lymphoma [37 cases], with higher male preponderance in the former [4.16:1] than in the latter [2.7:1]. Mixed cellularity was the largest subtype of HD [66.14%], whereas diffuse small cell [45.96%] dominated the NHL variants. We observed that HD was more common than NHL, and male to female ratio was higher in the former than in the latter in our study as compared to those reported from abroad. Further studies will confirm our findings