30 Years of Experience with Non-Hodgkin Lymphoma in Children and Adolescents: a retrospective cohort study

SUMMARY OBJECTIVE Describe the clinical and demographic characteristics of pediatric patients with non-Hodgkin's lymphoma (NHL) enrolled in a tertiary unit of Pediatric Hematology between 1982-2015. PATIENTS AND METHODS A retrospective cohort study of 140 patients aged 16 years or less with NHL. Demographic characteristics, data on diagnosis, and outcomes were analyzed. The overall survival (OS) analysis and stratification by the most frequent histological subtypes were performed using the Kaplan-Meier method. RESULTS One hundred and thirty-six patients with de novo NHL and four with NHL as a second malignancy were analyzed. The median age at diagnosis was 6.4 years (interquartile range, 4.2 to 11.1 years); 101 patients were males. Four patients had primary immunodeficiency, four had human immunodeficiency virus, two post-liver transplantation, and one had autoimmune lymphoproliferative syndrome. The most frequent histological type was NHL of mature B- cell (B-NHL-B; 67.1%), with Burkitt's lymphoma being the most frequent subtype, and lymphoblastic lymphoma (LBL, 21.4%). The main clinical manifestation at the diagnosis was abdominal tumors (41.4%). During the follow-up time, 13 patients relapsed, but five of them reached a second remission. Thirty-five patients died, and 103 remained alive in clinical remission. No contact was possible for two patients. The OS at 5 years was 74.5% (± 3.8%). The OS estimated for patients with LBL, NHL-B, and the remaining was 80.4%±7.9%, 72.8%±4.7%, and 74.5%±11%, respectively (P = 0.58). CONCLUSION Our results are comparable with cohorts from other middle-income countries.

RESUMO OBJETIVO Descrever as características clínicas e demográficas de pacientes pediátricos com linfoma não Hodgkin (LNH) em uma unidade terciária de Hematologia Pediátrica entre 1982-2015. PACIENTES E MÉTODOS Estudo de coorte retrospectivo de dados de prontuários de 140 pacientes com idade até 16 anos com LNH. Características demográficas e dados relativos ao diagnóstico e evolução foram analisados. A sobrevida global (SG) e estratificada pelos subtipos histológicos mais frequentes foi analisada pelo método de Kaplan-Meier. RESULTADOS Dados de 136 pacientes com LNH de novo e quatro com LNH como segunda neoplasia foram analisados. A mediana de idade ao diagnóstico foi 6,4 anos (intervalo interquartil: 4,2 a 11,1 anos); 101 pacientes eram meninos. Onze pacientes apresentavam imunodeficiência (quatro primária, quatro secundária ao vírus da imunodeficiência humana adquirida, dois pós-transplante hepático e um com síndrome linfoproliferativa autoimune). Os tipos histológicos mais frequentes foram o LNH de células B madura (LNH-B, 67,1% dos pacientes), sendo o linfoma de Burkitt o subtipo mais frequente, e o linfoma linfoblástico (LL, 21,4%). A principal manifestação clínica ao diagnóstico foi massa abdominal (41,4%). A mediana de seguimento dos sobreviventes foi 7,7 anos (intervalo interquartil: 3,3 a 10,9 anos). Treze pacientes recidivaram (cinco alcançaram segunda remissão clínica), 35 faleceram, 103 permanecem vivos em remissão completa e dois perderam o seguimento. A probabilidade de SG em cinco anos foi 74,5%±3,8%. Para os pacientes com LL, LNH-B e os demais, a SG foi 80,4%±7,9%, 72,8%±4,7% e 74,5%±11%, respectivamente (P=0,58). CONCLUSÃO Nossos resultados são comparáveis aos de outros países de renda média.

Infectious complications as a predictor of mortality in patients with Non-Hodgkin lymphoma receiving rituximab- containing chemotherapy

Abstract Background Rituximab is a monoclonal antibody that increases the disease-free and overall survival of patients with non-Hodgkin lymphoma (NHL) CD20+. The objective of this study is to describe the prevalence and spectrum of infections in patients with NHL receiving rituximab-containing chemotherapy and the impact on survival. Materials and Methods From January 2011 to December 2012, all patients diagnosed with NHL who received at least one dose of rituximab were included. Results During the study period, 265 patients received rituximab; 108 (40.8%) males; the mean age was 60 ± 15 years. There were 177 infections in 85 patients, being the most common febrile neutropenia (n = 38; 21.5%) and mucosal barrier injury-related infections (n = 28; 15.8%). In 88 events (49%), there was a microbiologic diagnosis, being bacterial infection the most frequent (39.6%), but tuberculosis (TB) was developed in 4 cases (1.5%; incidence rate 721/100,000 person-year). During follow-up, 71 patients died (27%); in 35 cases, it was related to infection. There were no differences in follow-up between those who died due to infection versus those who died from another cause (p = 0.188). Multivariate analysis for mortality showed that age >60 years, failure to achieve a complete response, and development of an infectious complication increased the risk of death. Conclusions It is important to perform a screening test for TB in all patients who will receive rituximab and maintain a constant monitoring to detect an infectious process and begin treatment as soon as possible.

Salvaged single-unit cord blood transplantation for 26 patients with hematologic malignancies not in remission

Treatments for patients with hematologic malignancies not in remission are limited, but a few clinical studies have investigated the effects of salvaged unrelated cord blood transplantation (CBT). We retrospectively studied 19 patients with acute leukemia, 5 with myelodysplastic syndrome (MDS with refractory anemia with excess blasts [RAEB]), and 2 with non-Hodgkin's lymphoma who received 1 CBT unit ≤2 loci human leukocyte antigen (HLA)-mismatched after undergoing myeloablative conditioning regimens between July 2005 and July 2014. All of them were in non-remission before transplantation. The infused total nucleated cell (TNC) dose was 4.07 (range 2.76-6.02)×107/kg and that of CD34+ stem cells was 2.08 (range 0.99-8.65)×105/kg. All patients were engrafted with neutrophils that exceeded 0.5×109/L on median day +17 (range 14-37 days) and had platelet counts of >20×109/L on median day +35 (range 17-70 days). Sixteen patients (61.5%) experienced pre-engraftment syndrome (PES), and six (23.1%) patients progressed to acute graft-versus-host disease (GVHD). The cumulative incidence rates of II-IV acute GVHD and chronic GVHD were 50% and 26.9%, respectively. After a median follow-up of 27 months (range 5-74), 14 patients survived and 3 relapsed. The estimated 2-year overall survival (OS), disease-free survival (DFS), and non-relapse mortality (NRM) rates were 50.5%, 40.3%, and 35.2%, respectively. Salvaged CBT might be a promising modality for treating hematologic malignancies, even in patients with a high leukemia burden.

Clinical characteristics, pathological distribution, and prognostic factors in non-Hodgkin lymphoma of Waldeyer's ring: nationwide Korean study

BACKGROUND/AIMS: In Asia, the incidence of non-Hodgkin lymphoma (NHL) has increased in recent decades. Waldeyer's ring (WR) is the most common site of NHL involving the head and neck. In this study, the pathological distribution of WR-NHL and its clinical features were analyzed retrospectively. METHODS: From January 2000 through December 2010, we analyzed the medical records of 328 patients from nine Korean institutions who were diagnosed with WR-NHL. RESULTS: The study group comprised 197 male and 131 female patients with a median age of 58 years (range, 14 to 89). The rate of localized disease (stage I/II) was 64.9%, and that of low-risk disease (low/low-intermediate, as defined by the International Prognostic Index) was 76.8%. Diffuse large B-cell lymphoma (DLBCL; 240 patients, 73.2%) was the most common pathologic subtype, followed by peripheral T-cell lymphoma (14 patients, 4.3%) and nasal NK/T-cell lymphoma (14 patients, 4.3%). WR-NHL occurred most frequently in the tonsils (199 patients, 60.6%). Extranodal involvement was greater with the T-cell subtype (20 patients, 42.5%) compared with the B-cell subtype (69 patients, 24.5%). Multivariate analyses showed that age > or = 62 years, T-cell subtype, and failure to achieve complete remission were significant risk factors for overall survival. CONCLUSIONS: DLBCL was found to have a higher incidence in Korea than those incidences reported by other WR-NHL studies. T-cell lymphoma occurred more frequently than did follicular lymphoma. T-cell subtype, age > or = 62 years, and complete remission failure after first-line treatment were significant poor prognostic factors for overall survival according to the multivariate analysis.

Intervenciones de enfermería en la atención del adulto con linfoma no hodgkin folicular (LNHF) / Nursing interventions in adult attention with Follicular Non-Hodgkin lymphoma (LNHF)

Introducción: Esta guía pone a disposición del personal de enfermería que labora en unidades médicas de tercer nivel de atención, las recomendaciones basadas en la mejor evidencia disponible para la toma de decisiones clínicas, las intervenciones de enfermería en la atención del adulto con Linfoma no Hodgkin Folicular. Método: Se realizó una búsqueda y revisión sistemática en la base de datos de PubMed, sitios específicos de Guías de Práctica Clínica y la biblioteca Cochrane; publicados entre 2002 y 2012, de los cuales se seleccionaron las fuentes con mayor puntaje obtenido en la evaluación de su metodología y las de mayor nivel en cuanto a gradación de evidencias y recomendaciones. Resultados: En la revisión sistemática fueron seleccionados 19 documentos para la elaboración de la guía. Se recomienda al profesional de enfermería fomentar la valoración del paciente identificando las necesidades físicas y emocionales del paciente y de la familia. Las intervenciones deben ser dirigidas antes, durante y después del tratamiento con enfasis en los cuidados de los efectos secundarios de la quimioterapia.Conclusiones: La incorporación de las recomendaciones en la práctica asistencial de enfermería podrá favorecer en la limitación del daño, disminución de comorbilidad y mejora en la calidad de vida en los pacientes mayores de 18 años con linfoma no Hodgkin folicular.

Introduction: This guide provides nursing staff working in medical units tertiary care recommendations based on the best evidence available for clinical decision making, nursing interventions in the care of adults with follicular Non-Hodgkin lymphoma.Method: A search and systematic review was performed in the PubMed database, specific sites Clinical Practice Guidelines and the Cochrane Library; were published between 2002 and 2012, of which the sources were selected with the highest score obtained in the evaluation of its methodology and higher level as to grading evidence and recommendations.Results: In the systematic review they were selected 19 documents for the development of the guide. It is recommended to encourage nurse patient assessment identifying the physical and emotional patient and family needs. Interventions should be directed before, during and after treatment with emphasis on the care of the side effects of chemotherapy.Conclusions: The incorporation of the recommendations in the nursing care practice may favor in limiting the damage, decreased morbidity and improved quality of life in patients over 18 years with follicular non-Hodgkin Lymphoma.

Introdução: Este guia fornece a equipe de enfermagem que trabalham em unidades médicas terciárias cuidar recomendações baseadas na melhor evidência disponível para a tomada de decisão clínica, intervenções de enfermagem no cuidado de adultos com Linfoma folicular não-Hodgkin.Método: Uma pesquisa e revisão sistemática foi realizada no banco de dados PubMed, sites específicos diretrizes de prática clínica e da Biblioteca Cochrane; foram publicados entre 2002 e 2012, dos quais as fontes foram selecionados com a maior pontuação obtida na avaliação de sua metodologia e nível superior quanto à classificação de evidências e recomendações.Resultados: Na revisão sistemática foram selecionados 19 documentos para o desenvolvimento do guia. Recomenda-se a incentivar a enfermeira avaliação do paciente identificar as necessidades físicas e emocionais do paciente e da família. As intervenções devem ser dirigidos antes, durante e após o tratamento com ênfase no cuidado dos efeitos colaterais da quimioterapia.Conclusões: A incorporação das recomendações na prática de cuidados de enfermagem pode favorecer em limitar os danos, diminuição da morbidade e melhor qualidade de vida em pacientes com mais de 18 anos com linfoma não-Hodgkin folicular.

Virus de la Inmunodeficiencia Humana y Linfoma en un Hospital Universitario de Corrientes Argentina / HIV and Lymphoma in a University Hospital in Corrientes Argentina

Los pacientes infectados por el HIV presentan una mayor morbimortalidad por cáncer como Linfoma y Sarcoma de Kaposi, constituyendo en este grupo entre la 2da y 3ra causa de muerte, a pesar de la TARV . Varios estudios han demostrado la disminución de infecciones oportunistas, Sarcoma de Kaposi, Linfoma primario cerebral,en menor proporción de otros Linfomas NoH como el Linfoma Burkit, y un aumento de neoplasias no asociados al SIDA como el Linfoma Hodgkin.Objetivo: Analizar las características clínicas, tratamiento, sobrevida de pacientes HIV seropositivos con linfoma,diagnosticados y tratados en el Servicio de Clínica Médica del Hospital Escuela José F. de San Martín en la ciudad de Corrientes- Argentina. Evaluar la prevalencia se seropositividad en nuestra población de pacientes con Linfoma, y determinar el pronóstico al momento del diagnóstico.Material y Método: Se realizó un estudio descriptivo observacional mediante un análisis retrospectivo de las historias clínicas de siete pacientes con diagnóstico de Linfoma y serología (+) para HIV, tratados en el servicio de Clínica Médica del Hospital Escuela de Corrientes desde Enero 2003 a Enero 2013.Resultados: Entre 76 casos de Linfoma, 18 fueron tipo Hodgkin y 58 tipo NH. Del total 7 tenían serología positiva para HIV (12,5%) ,1 de 18 con enfermedad de Hodgkin y 6 de 58 LNH, es decir 5.5% y 10.34% respectivamente...

Tendência das taxas de mortalidade por linfoma não-Hodgkin na Região Sudeste do Brasil, 1980-2007 / Trends in mortality rates from non-Hodgkin lymphoma in Southeast Brazil, 1980-2007

A mortalidade por linfoma não-Hodgkin vem diminuindo em vários países, porém, para o Brasil, as estimativas apontam 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 Informações sobre Mortalidade (SIM) e o Departamento de Informática do SUS (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 estatisticamente significativa em Belo Horizonte (Minas Gerais) 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 estatisticamente significativa no período inicial. Os resultados sugerem que o incremento observado entre 1980-2007 poderia ser resultante do crescimento das taxas de mortalidade entre 1980-1995, já que, no último período, não foram observadas tendências estatisticamente significativas nessas cidades.

Mortality rates from non-Hodgkin lymphoma (NHL) have declined in many countries in recent decades. However, mortality estimates for Brazil indicate an increase in these rates. This study aimed to analyze NHL mortality trends for 1980-2007 in individuals 20 years and older in State capitals in Southeast Brazil. Population data were obtained from the Mortality Information System and the Health Statistics Division of the Unified National Health System (DATASUS). Age-related mortality trends were analyzed using polynomial regression models. In the 60 and older age group, a statistically significant upward linear trend was observed for Belo Horizonte and São Paulo in 1980-2007. When analyzed in two different periods, 1980-1995 and 1996-2007, statistically significant increases in NHL mortality rates were only observed in the former period. These results suggest that the increase in 1980-2007 may have resulted from the rising mortality rates from 1980 to 1995, since no statistically significant trends were observed in the latter period.

Epidemiología del linfoma con compromiso de cabeza y cuello en el Centro Asistencial Sótero del Río / Epidemiology of lymphoma of the head and neck in the Centro Asistencial Sótero del Río

Introducción: Los linfomas son la neoplasia hematológica más frecuente del adulto, ocupando el tercer lugar de las neoplasias de cabeza y cuello (CyC). Se distinguen dos tipos, el linfoma de Hodgkin (LH) y el linfoma no Hodgkin (LNH), constituyendo dos entidades clínicas separadas. Objetivo: Evaluar las manifestaciones de los linfomas en la región de CyC, analizando las diferencias de presentación y comportamiento entre en LH y el LNH. Material y método: Estudio retrospectivo de todos los casos de linfomas de CyC en el Centro Asistencial Dr. Sótero del Roo (CASR) entre los años 2005 y 2009, documentando específicamente presentación clínica, factores de riesgo, el tipo de linfoma, diagnóstico histológico, localización anatómica, etapificación al momento del diagnóstico, tratamiento administrado, sobrevida. Resultados: Se incluyeron 80 casos de pacientes con linfoma de CyC en el estudio, 16 (20 por ciento) con LH y 64 (80 por ciento) con LNH. Se encontraron diferencias estadísticamente significativas entre ambos para la edad de diagnóstico, el compromiso extranodal, compromiso de mediastino, presencia de síntomas B y sobrevida. Conclusión: En el CASR la presentación de los linfomas es similar a la reportada mundialmente con diferencias similares entre LH y LNH.

Introduction: Lymphomas are the most common hematologic neoplasia in the adult and the third most common neoplasias of the head and neck (CyC). There are two types of lymphoma: Hodgkin and non-Hodgkin lymphomas, which constitute two unique clinical entities. Aim: To evaluate the clinical manifestations of lymphomas in the CyC region, analizing the diferences between the clinical presentation and behavior of LH and LNH. Material and method: This is an retrospective study of all the cases of lymphoma of CyC in the Centro Asistencial Dr. Sótero del Río (CASR) between the years 2005 and 2009, describing specifically the clinical presentation, risk factors, type of lymphoma, histologic diagnosis, anatomical localization, etapification at diagnosis, treatment received and survival. Results: Eighty cases of lymphoma of the CyC were included, 16 patients (20 percent) with LH and 64 patients (80 percent) with LNH. We found statistically significant differences between both groups for their age at diagnosis, extranodal disease, mediastinal compromise, presence of "B" symptoms and survival. Conclusion: in the CASR the clinical presentation of lymphomas is similar to that reported in the international literature, maintaining the unique characteristics for LH and LNH.

HIV-associated non-Hodgkin's lymphoma: Experience from a regional cancer center.

Aims : To analyze clinical features and survival in HIV-associated non-Hodgkin lymphoma (NHL) cases registered at Dr BRA Institute Rotary Cancer Hospital of AIIMS, New Delhi. Materials and Methods : We have retrospectively reviewed records of NHL patients registered, from January 2003 to July 2007 to analyze HIV-associated NHL. Results : Seven cases of HIV-associated NHL cases were identified. Age range was 14-56 years. Five were males. Baseline performance status (ECOG-PS) was >I in 6. Mean LDH was 409 U/L. Mean hemoglobin was 10.5 g% and mean CD4 count was 243/mm3 (range 18- 454). Three cases had nodal lymphoma and four had extra nodal lymphoma. No primary CNS (PCNSL) lymphoma was seen. All patients were of advanced stages and of intermediate to high-risk group based on international prognostic index (IPI). Six cases had high-grade NHL. None had CNS involvement. Five had B symptoms. HIV infection was diagnosed as part of NHL work-up in five patients. All patients received HAART. All were planned for chemotherapy with CNS prophylaxis. Protocols used were CVP, CHOP, R-CHOP or MCP-842. One patient received IFRT. Response : One patient achieved complete response (CR) and continues to be disease free, with 4.5 years of follow-up. Three cases achieved partial response (PR) and 2 had progressive disease (PD). Currently, three patients are on follow-up. Conclusions : These NHL are of higher grade and advanced stage. Response and tolerance to chemotherapy is poor. Appropriate supportive care and CNS prophylaxis might improve outcome. We need to improve epidemiological data collection system in this part of world. With HAART, the goal of therapy is durable CR rather than palliation.

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...

Linfoma não-Hodgkin na infância: características clínico-epidemiológicas e avaliação de sobrevida em um único centro no Nordeste do Brasil / Non-Hodgkin's lymphoma in childhood: clinical and epidemiological characteristics and survival analysis at a single center in Northeast Brazil

OBJETIVO: Descrever o perfil clínico-epidemiológico dos pacientes portadores de linfoma não-Hodgkin diagnosticados no Serviço de Oncologia Pediátrica do Instituto Materno-Infantil Professor Fernando Figueira em um período de 9 anos, bem como descrever sobrevida e possíveis associações prognósticas com as variáveis clínico-epidemiológicas estudadas. MÉTODOS: Estudo descritivo de corte transversal, realizado através da análise dos prontuários de 110 pacientes com linfoma não-Hodgkin admitidos na instituição no período de maio de 1994 a maio de 2003. As sobrevidas global e livre de doença foram analisadas através da técnica de Kaplan-Meier, e o teste de log rank foi utilizado para avaliar diferenças entre os grupos. RESULTADOS: A idade média foi de 6,1 anos, e a relação masculino-feminino, 2,4:1. O subtipo histológico mais freqüente foi o linfoma de Burkitt. A maioria dos pacientes foi diagnosticada em estádio III e IV da classificação de Murphy e provinha da zona rural. Renda familiar per capita inferior a 1/2 salário mínimo foi observada em 36,4 por cento, e analfabetismo materno, em 12,7 por cento dos casos. A probabilidade de sobrevida global e livre de doença aos 5 anos foi de 70±4 por cento e 68±4 por cento, respectivamente. Nenhuma das variáveis clínico-epidemiológicas analisadas mostrou associação estatística significante com a probabilidade de sobrevida dos pacientes (p > 0,05). CONCLUSÃO: Observamos incidência mais elevada do subtipo Burkitt e de crianças acometidas em idade mais jovem quando comparada à descrita em literatura estrangeira. A sobrevida observada aproximou-se dos resultados descritos pelos principais grupos cooperativos de tratamento de câncer infantil. As variáveis clínico-epidemiológicas analisadas não apresentaram associação prognóstica estatística significante.

OBJECTIVE:To describe the clinical and demographic characteristics of non-Hodgkin's lymphoma patients diagnosed at the Pediatric Oncology Unit at the Instituto Materno-Infantil Professor Fernando Figueira (IMIP) over a 9-year period, and also to describe their survival rates and possible associations between the survival rates and the clinical and demographic characteristics analyzed in the study. METHODS:This was a cross-sectional study. Data were collected by a retrospective review of the charts of all 110 patients admitted to our unit during the period of May 1994 through May 2003. Probability of survival was calculated in accordance with the techniques of Kaplan-Meier, using log rank to evaluate differences between the groups. RESULTS:The average age was 6.1 years. The male/female ratio was 2.4:1. The most frequent histological subtype was Burkitt's lymphoma. The majority of patients had been diagnosed with advanced disease (stage III or IV of Murphy's Classification) and was from rural areas. Family income per capita was lower than 1/2 minimum wage in 36.4 percent of cases; maternal illiteracy was observed in 12.7 percent of cases. The 5-year overall survival and disease-free survival rates were 70±4 percent and 68.4±4 percent, respectively. None of the clinical-demographic characteristics had a significant association with the probability of survival (p > 0.05). CONCLUSION: Children admitted to the IMIP seemed to be affected by non-Hodgkin lymphoma at a younger age, with a higher incidence of Burkitt's lymphoma and with survival rates similar to those described in the literature of developed countries. No clinical demographic characteristics had a statistically significant association with prognosis.

Linfomas del tubo digestivo y glándulas anexas en pacientes con SIDA: serie de casos / Non-Hodgkin's lymphomas of the digestive tract and anexal glands in AIDS patients

Introducción: el linfoma no Hodgkin (LNH) es la segunda neoplasia más frecuente en pacientes con SIDA. Una de las principales características de los LNH asociados con el SIDA es la alta frecuencia de compromiso extranodal, incluyendo el tracto astrointestinal, como manifestación inicial de la neoplasia. Métodos: se analizaron de manera retrospectiva las características epidemiológicas, clínicas, virológicas, inmunológicas e histopatológicas de 8 pacientes con diagnóstico de linfomas del tracto gastrointestinal y glándulas anexas (hígado y parótida) asistidos en la División de VIH- /SIDA del Hospital de Enfermedades Infecciosas F J Muñiz de Buenos Aires, Argentina, entre enero de 1997 y diciembre de 2004. Todos los pacientes fueron evaluados por tomografía computarizada y examen de la médula ósea, además de los exámenes endoscópicosde diagnóstico. Resultados: todos los pacientes fueron varones; 4 eran heterosexuales, 2 homosexuales, uno hemofílico y uno adicto a drogas endovenosas. Tres pacientes presentaron coinfección por el virus de la hepatitis C. La mediana de edad fue de 42 años y la mediana de linfocitos T CD4 + fue de 87 células/uL al momento del diagnóstico de la neoplasia. Ningún paciente recibía TARGA al momento del diagnóstico del linfoma. La incidencia global de linfomas (primarios del sistema nervioso central, no Hodgkin sistémicos y de Hodgkin) durante el período de estudio fue de 2,9% (54 casos); 17 pacientes (32%) tuvieron diagnóstico de LNH sistémicos; 10 (58,8%) de ellos tuvieron presentación extranodal al momento del inicio de las mani manifestaciones clínicas y 8 (80%) comprometieron el tubo digestivo y las glándulas anexas (parótida, cavum, esófago, estómago, duodeno, colon derecho en 2 casos e hígado en el restante), como LNH primarios, de alto grado y de fenotipo "B". Todos los pacientes presentaronsíntomas "B" al momento del diagnóstico. De acuerdo con los criterios de estadificación de Lugano, 4 se encontraban en el estadio...

Background: Non-Hodgkin's lymphoma (NHL) is the second most common neoplasm among patients with AIDS. One of the major clinical characteristics of AIDS-associated NHL is the high frequency of extranodal involvement, including the gastrointestinaltract, at initial presentation. Methods: From January 1997 to December 2004, 8 cases of NHL of the digestive tract and anexal glands (liver and parotid gland) were observed at the HIV/AIDS division of the Infectious Diseases F J Muñiz Hospital from Buenos Aires,Argentina. All patients were staged by computed tomography scanning and bone marrow examination, in addition to the endoscopic evaluation. Results: All patients were males; 4 were heterosexual, 2 homosexual, and 1 were a hemophilic and an intravenous drug abuser. The median age was 42 years and the median CD4 T cell count was 87 cells/uL at the time of the diagnosis of neoplasm. No patient was receiving highly active antiretroviral therapy (HAART) at lymphoma diagnosis. The global incidence of AIDS-associated lymphomas (central nervous system lymphomas, non-Hodgkin lymphomas and Hodgkin lymphoma) during the time of study was 2,9% (54 cases); 17 patients(32%) had diagnosis of systemic NHL; 10 (58,8%) of them were extranodal at the onset of clinical symptoms and 8 (80%) involvement the digestive tract and anexal glands (parotid gland, cavum, esophagus, stomach, duodenum, the right colon in 2 patients and the liver), as primary NHL of high grade and "B" phenotype. All patients presented "B" symptoms at the time of diagnosis. Primary duodenal lymphoma was the only Burkittlymphoma of this serie and we detected the Epstein-Barr virus genome in the biopsy smears of this tumor and in the hepatic lymphoma. Four patients were treated with systemic chemotherapy with granulocytic growth factor support plus highly active antiretroviral therapy (HAART); 2 of them (cavum and one of the colon) had a prolonged survival with immune reconstitution during 5 and 6...

Resultados a largo plazo del tratamiento con quimioterapia y radioterapia en linfoma no Hodgking localizado, de grados intermedio y alto / Long term results for intermediate and high grade localized non Hodgkin lymphoma, treated with chemotherapy and radiotherapy

Background: Treatment of intermediate and high grade non-Hodgkin lymphoma (NHL) includes chemotherapy with or without radiotherapy, depending on the clinical stage. The standard treatment for advanced NHL is 8 cycles of combined chemotherapy, cyclophosphamide, adriamicin, vincristine and prednisone (CHOP). Patients presenting with localized disease are treated with fewer chemotherapy cycles and involved field radiotherapy, with good results. Aim: To evaluate the treatment results including overall survival (OS) and event-free survival (EFS) in localized aggressive NHL patients treated at the Pontificia Universidad Católica de Chile, Clinical Hospital. Patients and Methods: Retrospective analysis of all patients with Ann Arbor stages I and II referred to the hematology and radiotherapy clinic between 1998 and 2003. OS and EFS analysis was made according to the Kaplan and Meier method. Log-rank and Cox methods were used for univariate and multivariate analyses, respectively. Chemotherapy and radiotherapy toxicities were scored according to World Health Organization (WHO) and Radiation Therapy Oncology Group (RTOG) scales, respectively. Results: 39 patients (20 men), aged between 20 to 85 years, were the source for this study. The average follow-up was 51 months (range 6-115). The 5 years OS and EFS were 72,4 percent and 63,3 percent, respectively. On univariate analysis, age over 60 was the only variable that affected negatively OS and EFS. Acute toxicity caused by chemotherapy and radiotherapy was uncommon. Conclusions: Age over 60 was the only independent variable associated with poor prognosis. The number of chemotherapy cycles and the drug combination did not influence the results. These results support the usefullness of a shortened chemotherapy regimen plus involved field radiotherapy.

Curative resection plus adjuvant chemotherapy for early stage primary gastric non-Hodgkin's lymphoma: a retrospective study with emphasis on prognostic factors and treatment outcome

RACIONAL: A terapêutica do linfoma não-Hodgkin gástrico primário é controversa, com defensores da extirpação cirúrgica, da radioterapia e quimioterapia isoladas ou combinadas, especialmente em relação aos estádios mais iniciais. OBJETIVOS: Analisar as características clínico-patológicas e os resultados do tratamento nos doentes operados no Serviço de Gastroenterologia Cirúrgica do Hospital do Servidor Público Estadual, São Paulo, SP, com linfoma gástrico primário em estádio inicial. Realizar revisão da literatura, destacando os aspectos diagnósticos, fatores prognósticos e o papel das diferentes modalidades de tratamento. MÉTODO: Dezesseis doentes com linfoma gástrico primário no estádio inicial foram submetidos ao tratamento cirúrgico curativo. Idade, sexo, localização, tamanho, tipo de operação, número de lesões, profundidade da invasão, tipo histológico de acordo com a classificação de Kiel, comprometimento linfonodal, estádio pela classificação de Ann Arbor modificada por Musshoff e Schmidt-Vollmer, grau histológico, margens, terapia adjuvante, evolução e sobrevivência. RESULTADOS: Dez (62,5%) doentes foram submetidos a gastrectomia subtotal e seis (37,5%) a gastrectomia total. A maioria (9/56,2%) das lesões estava localizada no antro. Lesões únicas (10/62,5%) foram mais freqüentes que as múltiplas (6/37,5%). Treze doentes (81,2%) foram classificados no estádio IE e três (18,7%) no estádio IIE1. Dez (62,5%) enfermos apresentaram linfoma gástrico primário de baixo grau e seis (37,5%) de alto grau. Os tipos histológicos mais freqüentes foram o linfoplasmocítico citóide (4/25.0%) e o centroblástico (4/25,0%). Dez doentes (62,5%) receberam tratamento adjuvante (quimioterapia e/ou radioterapia). Nove enfermos (56,2%), todos no estádio IE, atingiram sobrevivência maior que 5 anos e oito (50,0%) receberam tratamento adjuvante. Dois (12,5%) doentes no estádio IIE1 tiveram recidiva peritonial e faleceram 3,0 anos e 3,5 anos após suas respectivas operações. A média global de sobrevivência foi de 42,5 meses...

Clinicopathologic Characteristics and Therapeutic Outcomes of Primary Gastrointestinal Non-Hodgkin's Lymphomas in Central Anatolia, in Turkey

Primary gastrointestinal lymphoma is a common presentation of non-Hodgkin's lymphoma. The main controversy arises when many aspects of its classification and management are under discussion, particularly regarding roles for surgical resection. The aim of this study was to evaluate clinicopathologic characteristics and the therapeutic outcome of primary gastrointestinal non-Hodgkin's lymphoma. We carried out a retrospective analysis of 74 patients who were presented to our center with histopathological diagnosis of primary gastro-intestinal non-Hodgkin's lymphoma between 1990 and 2001. All patients have been staged according to Lugano Staging System. For histopathological classification, International Working Formulation was applied. The treatment choice concerning the surgical or non-surgical management was decided by the initially acting physician. Treatment modalities were compared using the parameters of age, sex, histopathological results, stage, and the site of disease. Of the 74 patients, 31 were female and 43 were male, with a median age of 49 years (range 15-80). The stomach was the most common primary site and was seen in 51 of 74 patients (68.9%). The intermediate and high grade lymphomas constituted 91.9% of the all cases. In a median follow-up of 29 months (range 2-128), 20 out of 74 patients died. There was a three year overall survival rate in 65.4% of all patients. The three year overall survival rate was better in stage I and II1 patients who were treated with surgery plus chemotherapy (+/-RT) than those treated with chemotherapy alone (93.7% vs. 55.6%, p<0.05). The stage and presence of B symptoms affected the disease free survival and overall survival significantly, but the histopathologic grade only affected the overall survival. On the basis of these results, we suggest that surgical resection is necessary before chemotherapy in early stage (stage I and II1) patients with gastrointestinal non-Hodgkin's lymphomas because of the significant survival advantage it would bring to the patient.

Eleven-Year Experience of Low Grade Lymphoma in Korea (Based on REAL Classification)

Low grade lymphomas are malignancies of predominantly small lymphocytes that typically have long median survival periods due to low proliferative rates. It is considered an indolent disease, but patients with low grade lymphoma can almost never be cured with conventional treatment. New low-grade lymphoma entities have been classified by the International Lymphoma Study Group (ILSG) and are also categorized into the Revised European American Lymphoma (REAL) classification. The REAL classification utilizes a multiparameter definition of clinico-pathologic and biologic entities. According to this classification, we investigated the incidence, various clinical characteristics, treatment outcome and prognostic factors of low grade lymphoma. Many clinical characteristics of low grade lymphoma in Korea differed from those of Western countries, especially in the incidence, therapeutic outcome and prognostic factors. In Korea, although the general incidence of low grade lymphoma is relatively low, the relative number of mucosa-associated lymphoid tissue lymphoma (MALToma) is very high, and the overall survival rate is better than that reported of Western countries. Thus, further investigation on treatment outcome and prognosis of low grade lymphoma entities, other than mucosa-associated lymphoid tissue lymphoma, are warranted.

Expression of cyclin-dependent kinase inhibitor p27kip1 in malignant lymphomas

p27kip1 is a cyclin-dependent kinase inhibitor that regulates progression from G1 into S phase. Aberrations in cell cycle control are often observed in tumors d might even be necessary in tumor development. Recent reports showed that low 7kip1 expression is associated with poor prognosis in several tumors and ukemia. To investigate the expression of p27kip1 in malignant lymphomas and ucidate the role of p27kip1 as a possible prognostic indicator, the authors rformed an immunohistochemical staining of p27kip1 correlated with Ki-67 belling index and clinical parameters. p27kip1 expression was reduced variably most malignant lymphomas and inversely correlated with Ki-67 labelling index +AD0-0.0151). Regarding chemotherapeutic response, p271kip1 expression in the mplete remission group showed statistically significant difference in pression compared to the progressive disease group (p+AD0-0.0021). There were gnificant differences in survival between cases with low and high p27kip1 pression (p+AD0-0.0071). In a multivariate Cox analysis, p27kip1 expression was dependent prognostic factors as well as other known prognostic factors cluding age, grade, stage and chemotherapeutic response. In conclusion, the udy suggests that reduced expression of p27kip1 protein may play a role in the thogenesis and biologically aggressive behavior of malignant lymphomas.

Expression of cyclin-dependent kinase inhibitor p27kip1 in malignant lymphomas

p27kip1 is a cyclin-dependent kinase inhibitor that regulates progression from G1 into S phase. Aberrations in cell cycle control are often observed in tumors d might even be necessary in tumor development. Recent reports showed that low 7kip1 expression is associated with poor prognosis in several tumors and ukemia. To investigate the expression of p27kip1 in malignant lymphomas and ucidate the role of p27kip1 as a possible prognostic indicator, the authors rformed an immunohistochemical staining of p27kip1 correlated with Ki-67 belling index and clinical parameters. p27kip1 expression was reduced variably most malignant lymphomas and inversely correlated with Ki-67 labelling index +AD0-0.0151). Regarding chemotherapeutic response, p271kip1 expression in the mplete remission group showed statistically significant difference in pression compared to the progressive disease group (p+AD0-0.0021). There were gnificant differences in survival between cases with low and high p27kip1 pression (p+AD0-0.0071). In a multivariate Cox analysis, p27kip1 expression was dependent prognostic factors as well as other known prognostic factors cluding age, grade, stage and chemotherapeutic response. In conclusion, the udy suggests that reduced expression of p27kip1 protein may play a role in the thogenesis and biologically aggressive behavior of malignant lymphomas.

Linfoma centrofacial: un análisis retrospectivo, clínico, patologíco y terapeutico de 10 casos / Centrofacial linfoma: a therapeutic pathological clinical retrospective analysis of ten cases

Se comunican 10 casos de linfoma centrofacial estudiados de manera retrospectiva entre 1995 y 1999, que correspondieron a 6.6 por ciento de todos los linfomas diagnosticasos durante este periodo; cuatro de los 10 pacientes fueron del sexo masculino, con mediana de edad de 59 años 89 a 87 años). En dos pacientes se localizó en el paladar, en dos se afectaron antro nasal y senos paranasales, En otros dos casos se afectaron paladar y nariz, y en dos más al maxilar; uno se localizó en el párpado y el tabique nasal y otro en el paladar y la amígdala derecha. Todos se mostraron como lesiones expansivas, mayores de 4 cm de diámetro (4.5 a 7 cm). Histopatológicamente, tres fueron de alto grado de malignidad (angiocéntricos) y siete de grado intermedio. En un caso se relacionó con infección por VIH. Según su estadio clínico al momento del diagnóstico, siete fueron estadio I; dos estadio II y uno estadio IV por infiltración asintomática de médula ósea. Todos recibieron quimioterapia combinada con ciclofosfamida, doxorrubicina, vincristina y prednisona cada 21 a 28 días, durante seis ciclos. Se logró respuesta completa en cuatro de los pacientes, respuesta parcial en dos y no hubo respuesta en cuatro enfermos. Fallecieron cuatro pacientes: dos por infección grave uno por actividad tumoral y uno por toxicidad a quimioterapia. La supervivencia libre de enfermedad es de 39 por ciento y la supervivencia total de 56 por ciento a 36 meses. Nuestros resultados terapéuticos son similares a lo informado en la literatura; consideramos que ante un linforma centrofacial es obligado realizar fenotipo inmunológico de las células linfomatosas y análisis exhaustivos para detectar genoma de virus linfotrópicos en el tejido tumoral, en virtud de sus implicaciones pronósticas, y un mayor número de estudios prospectivos y controlados que permitan conocer las características clínicas, inmunofenotípicas y de asociación con infecciones virales en pacientes mestizos mexicanos y establecer el mejor esquema terapéutico para las particularidades de nuestro país