No description available.

Classic Hodgkin Lymphoma (cHL) has an annual incidence of 2.4 cases per 100 000 population in the UK, and is one of the most common malignancies diagnosed in young adults aged 15 to 34. The majority of younger patients have a good long-term outcome with between 80 and 90% disease-specific survival but cHL also affects older adults in whom the prognosis is significantly poorer. The role of tumour-associated macrophages (TAM) in cHL has gained much interest, with several studies reporting an association between high numbers of CD68-positive TAM and poor prognosis. There is also a question over the prognostic significance of Epstein-Barr Virus (EBV) infection which is implicated in up to 50% of cHL cases in developed countries. Published data suggests that EBV positivity in elderly patients may be associated with a poorer outcome, whereas in younger adults may be of prognostic benefit. Differences related to age are of interest particularly as an age-related decline in immunity has been linked with the development of certain subtypes of Non-Hodgkin Lymphoma in older patients. In a retrospective study, two separate cohorts of patients with cHL were examined with the aim of identifying: • Differences in the cellular composition of the tumour microenvironment in cHL which has arisen in young and elderly adult patients; • Differences in the cellular composition of the tumour microenvironment in cHL associated with or without EBV infection; • Factors within the tumour microenvironment which may influence prognosis and may be targeted for novel treatments. One group consisted of patients aged between 15 and 34 years at diagnosis and the other, of those aged 60 or over at presentation. Tissue obtained at the time of diagnosis was examined with regard to a number of factors related to the malignant cells and the surrounding microenvironment, including the number and phenotype of macrophages, the number of plasmacytoid dendritic cells and the number of malignant Hodgkin Reed-Sternberg (HRS) cells and non-malignant ‘background’ cells undergoing apoptosis. Comparisons were made between the two age groups, also taking into account the EBV-status of tumours, cHL subtype and gender. Results confirmed the current understanding that EBV-positive cHL is more common in older patients and has a strong, but not exclusive, association with the MCHL subtype. In addition, a strong link between young males and EBV-positive disease was shown. Macrophages were found to vary between the two age groups, in number and phenotype and there were clear differences associated with the presence or absence of EBV infection. While no definite link with outcome and macrophages was identified it was apparent that the implications of macrophages in the tumour microenvironment may differ between the two age groups. The number of apoptotic cells correlated closely with the number of macrophages and in the young the number of HRS cells was associated with prognosis. Investigation of the tumour microenvironment is complex and caution is needed in interpreting studies which do not differentiate between patients according to age, as tumour characteristics may have variable implications in different age groups. In this thesis a number of clinicopathological differences were identified between the two age groups. These point to the need for further larger studies to delineate how such age-related differences may or may not be associated with immune function and how this information could be translated into treatments to improve outcomes.

Pacientes com linfoma de Hodgkin (LH) tratados com poliquimioterpia com adriamicina, bleomicina, vincristina e doxorrubicina (ABVD) apresentam resposta terapêutica distinta. Para aprimorar a avaliação prognóstica e a abordagem terapêutica em LH objetivamos avaliar o valor prognóstico da PET-FDG após 2 ciclos de ABVD (PET2) em pacientes com LH. Foram incluídos nesse estudo prospectivo 115 pacientes com diagnóstico recente de LH no período de agosto de 2005 a dezembro de 2007. Os pacientes foram estadiados com exame clínico, laboratorial, tomografia computadorizada e PET-FDG (PET0). Todos os pacientes foram tratados com ABVD e aqueles com massa tumoral extensa foram tratados com radioterapia associada. Após dois ciclos de ABVD os pacientes foram submetidos a PET2. Nenhum tratamento foi alterado baseado na PET2. Foi avaliado o valor prognóstico dos fatores clínicos, Índice Prognóstico Internacional (IPI) e PET2 em relação à sobrevida livre de eventos (SLE) em três anos. Dos 104 pacientes que foram avaliados, 82 atingiram remissão completa e 22 pacientes apresentaram falha de tratamento durante a mediana de 36 meses de acompanhamento. A SG e SLE em três anos foi de 94,2% e 74,2% respectivamente. A SLE em três anos da PET2 positiva foi de 54,3%, enquanto da PET2 negativa foi de 90,5% (p< 0.001). Na análise de subgrupos de pacientes com estádio precoce, avançado, IPI baixo e alto risco, a PET2 também apresentou correlação estatisticamente significativa com o prognóstico. Concluímos que a PET2 é o melhor fator prognóstico independente na avaliação de pacientes com LH / Patients with Hodgkin lymphoma (HL) treated with poliquimioteraphy with adriamycin, bleomycin, vincristine and doxorubicin (ABVD) have distinct therapeutic response. In order to improve the prognostic assessment and therapeutic approach in HL we have evaluated the prognostic value of FDG-PET after 2 cycles of ABVD (PET2). Were included in this prospective study 115 patients with newly diagnosed LH in the period of August 2005 to December 2007. The patients were staged with physical examination, laboratory, CT and PET-FDG (PET0). All patients were treated with ABVD and those with extensive tumor were treated with radiotherapy associated. After two cycles of ABVD patients underwent PET2. No treatment was changed based on PET2. We assessed the prognostic value of clinical factors, international prognostic score (IPS) and PET2 in relation to event-free survival (EFS) in three years. Of the 104 patients who finalized the evaluation, 82 achieved complete remission and 22 patients experienced treatment failure during the median of 36 months of follow-up. The EFS at three years was 74.2%. EFS in three years of PET2 positive was 54.3%, while the PET2 negative was 90.5% (p <0.001). In subgroup analysis of patients with early stage, advanced, low and high risk IPS, PET2 also showed significant correlation with the prognosis. We conclude that the PET2 is the best independent prognostic factor in the evaluation of overall patients with LH, or in subgroups of early, advance; low and high risk of HL

No description available.

Pacientes com linfoma de Hodgkin (LH) tratados com poliquimioterpia com adriamicina, bleomicina, vincristina e doxorrubicina (ABVD) apresentam resposta terapêutica distinta. Para aprimorar a avaliação prognóstica e a abordagem terapêutica em LH objetivamos avaliar o valor prognóstico da PET-FDG após 2 ciclos de ABVD (PET2) em pacientes com LH. Foram incluídos nesse estudo prospectivo 115 pacientes com diagnóstico recente de LH no período de agosto de 2005 a dezembro de 2007. Os pacientes foram estadiados com exame clínico, laboratorial, tomografia computadorizada e PET-FDG (PET0). Todos os pacientes foram tratados com ABVD e aqueles com massa tumoral extensa foram tratados com radioterapia associada. Após dois ciclos de ABVD os pacientes foram submetidos a PET2. Nenhum tratamento foi alterado baseado na PET2. Foi avaliado o valor prognóstico dos fatores clínicos, Índice Prognóstico Internacional (IPI) e PET2 em relação à sobrevida livre de eventos (SLE) em três anos. Dos 104 pacientes que foram avaliados, 82 atingiram remissão completa e 22 pacientes apresentaram falha de tratamento durante a mediana de 36 meses de acompanhamento. A SG e SLE em três anos foi de 94,2% e 74,2% respectivamente. A SLE em três anos da PET2 positiva foi de 54,3%, enquanto da PET2 negativa foi de 90,5% (p< 0.001). Na análise de subgrupos de pacientes com estádio precoce, avançado, IPI baixo e alto risco, a PET2 também apresentou correlação estatisticamente significativa com o prognóstico. Concluímos que a PET2 é o melhor fator prognóstico independente na avaliação de pacientes com LH / Patients with Hodgkin lymphoma (HL) treated with poliquimioteraphy with adriamycin, bleomycin, vincristine and doxorubicin (ABVD) have distinct therapeutic response. In order to improve the prognostic assessment and therapeutic approach in HL we have evaluated the prognostic value of FDG-PET after 2 cycles of ABVD (PET2). Were included in this prospective study 115 patients with newly diagnosed LH in the period of August 2005 to December 2007. The patients were staged with physical examination, laboratory, CT and PET-FDG (PET0). All patients were treated with ABVD and those with extensive tumor were treated with radiotherapy associated. After two cycles of ABVD patients underwent PET2. No treatment was changed based on PET2. We assessed the prognostic value of clinical factors, international prognostic score (IPS) and PET2 in relation to event-free survival (EFS) in three years. Of the 104 patients who finalized the evaluation, 82 achieved complete remission and 22 patients experienced treatment failure during the median of 36 months of follow-up. The EFS at three years was 74.2%. EFS in three years of PET2 positive was 54.3%, while the PET2 negative was 90.5% (p <0.001). In subgroup analysis of patients with early stage, advanced, low and high risk IPS, PET2 also showed significant correlation with the prognosis. We conclude that the PET2 is the best independent prognostic factor in the evaluation of overall patients with LH, or in subgroups of early, advance; low and high risk of HL

<p>The relationship between Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL) is not entirely elucidated and a clonal relation may be present more often than previously believed. Mechanisms of tumour progression and resistance to therapy are poorly understood.</p><p>Between 1974 and 1994 all individuals in Sweden with both HL and NHL were identified. Thirty-two cases were studied using clinical, histopathological and immunohistochemical methods. The second lymphoma often appeared in an aggressive clinical form and a significant correlation between the expression of p53 and LMP-1 in the first and second lymphoma was demonstrated.</p><p>The treatment outcome for 307 patients with advanced stages of HL, in an unselected population was in accordance with the treatment results of large centres world-wide. Some patients were successfully selected for a shorter chemotherapy-regimen without inferior treatment results.</p><p>In 124 patients with relapse, the survival of those primarily treated with radiotherapy according to the National guidelines was in accordance with the survival of patients of initially advanced stages. A worse outcome was found for those who received both chemotherapy and radiotherapy initially, probably because of a higher frequency of bulky disease in this group. </p><p>Immunohistochemical analysis of the tumour suppressor protein p53 and retinoblastoma protein (Rb) of paired samples at diagnosis and at relapse in 81 patients did not reveal any specific staining pattern affecting survival.</p><p>A novel B-cell line (U-2932) was established from a patient with a diffuse large B-cell lymphoma previously treated for advanced stage and subsequent relapses of HL. An identical rearranged IgH gene was demonstrated in tumour cells from the patient and in U-2932. A p53 point mutation was detected and over-expression of the p53 protein was found. A complex karyotype with high-level amplifications of the chromosomal regions 18q21 and 3q27, i.e. the loci for <i>bcl-2</i> and <i>bcl-6</i> were demonstrated. </p>

The relationship between Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL) is not entirely elucidated and a clonal relation may be present more often than previously believed. Mechanisms of tumour progression and resistance to therapy are poorly understood. Between 1974 and 1994 all individuals in Sweden with both HL and NHL were identified. Thirty-two cases were studied using clinical, histopathological and immunohistochemical methods. The second lymphoma often appeared in an aggressive clinical form and a significant correlation between the expression of p53 and LMP-1 in the first and second lymphoma was demonstrated. The treatment outcome for 307 patients with advanced stages of HL, in an unselected population was in accordance with the treatment results of large centres world-wide. Some patients were successfully selected for a shorter chemotherapy-regimen without inferior treatment results. In 124 patients with relapse, the survival of those primarily treated with radiotherapy according to the National guidelines was in accordance with the survival of patients of initially advanced stages. A worse outcome was found for those who received both chemotherapy and radiotherapy initially, probably because of a higher frequency of bulky disease in this group. Immunohistochemical analysis of the tumour suppressor protein p53 and retinoblastoma protein (Rb) of paired samples at diagnosis and at relapse in 81 patients did not reveal any specific staining pattern affecting survival. A novel B-cell line (U-2932) was established from a patient with a diffuse large B-cell lymphoma previously treated for advanced stage and subsequent relapses of HL. An identical rearranged IgH gene was demonstrated in tumour cells from the patient and in U-2932. A p53 point mutation was detected and over-expression of the p53 protein was found. A complex karyotype with high-level amplifications of the chromosomal regions 18q21 and 3q27, i.e. the loci for bcl-2 and bcl-6 were demonstrated.

Made available in DSpace on 2015-07-22T20:50:12Z (GMT). No. of bitstreams: 0 Previous issue date: 2010-08-25 / Os antígenos cancer/testículo (CTAs) são considerados promissores alvos para abordagens de imunoterapia em câncer devido à sua alta imunogenicidade e padrão de expressão praticamente restrito a tecidos tumorais (estão também expressos em células germinativas do testículo, placenta e ovário fetal). Apesar do padrão de expressão dos CTAs estar bem estabelecido em carcinomas, pouco se sabe sobre a expressão desses antígenos em neoplasias linfóides como Linfomas de Hodgkin (LH) e Linfomas não-Hodgkin (LNH). Objetivo: Avaliar o potencial desses antígenos específicos tumorais como candidatos à imunoterapia em linfomas, através da análise de expressão protéica dos CTAs e avaliação da resposta imune humoral espontânea contra esses antígenos, correlacionando os achados com os dados clínicos e prognóstico. Métodos: Três blocos de Tissue Microarray (TMA) foram construídos a partir de 38 amostras teciduais de LH clássico e 106 de LNH obtidos nos arquivos do Departamento de Anatomia Patológia da UNIFESP. As lâminas de TMA foram submetidas a um painel de imunohistoquímica para 9 CTAs, a saber: MAGE-A1, MAGE-A3, MAGE-A4, MAGE-A10, CT7, CT10, NY-ESO-1, LAGE e GAGE. A avaliação da imunidade humoral espontânea foi realizada em 97 amostras de soro de pacientes com LNH ao diagnóstico (59 dos quais foram incluídos na casuística do TMA), utilizando-se a técnica de ELISA em um painel mais amplo de CTAs (MAGEA1, MAGE-A3, MAGE-A4, MAGE-A10, NY-ESO-1, CT7, CT10, CT24, CT45, CT46, CT47, CT63, CT83, SSX-1, SSX-2, SSX-4, LAGE-1, GAGE-2, SAGE-1, XAGE-1). Resultados: De forma global, houve baixa expressão de CTAs nas amostras analisadas, visto que apenas 21,1% das amostras de LH e 11,3% das amostras de LNH apresentaram positividade para pelo menos 1 dos CTAs incluídos no painel de imunohistoquímica. MAGE-A (18,4%) e CT7 (13,2%) foram os CTAs mais frequentemente expressos em LH, enquanto MAGE-A (6,6%), GAGE (5,7%) e NY-ESO-1 (4,8%) foram os mais expressos em LNH. Apesar de não ter sido encontrada diferença estatisticamente significante na expressão de CTAs entre os subgrupos clínicos de LH, houve maior frequência de positividade nos pacientes com estadiamento avançado (28,6%), comparado àqueles com estadiamento inicial (11,8%). Nos subgrupos clínicos de LNH, a frequência de expressão de CTAs foi maior nos linfomas agressivos (14,9%) em relação aos indolentes (3,1%), nos linfomas difusos de grandes células B (LDGCB) (16,1%) comparado aos não-LDGCB (6,0%) e no subgrupo que não atingiu resposta completa (15,0%) comparado àqueles que obtiveram resposta completa (6,8%). Entretanto, as diferenças não foram estatisticamente significantes em nenhum desses subgrupos. Um achado inesperado no presente estudo foi a expressão mais frequente de CTAs no subgrupo de LNH com estadiamento inicial (I e II) comparado ao subgrupo com estadiamento avançado (III e IV). Apesar da diferença encontrada na análise de sobrevida entre o grupo de pacientes que não apresentaram expressão de CTA (sobrevida mediana de 65 meses) e aqueles que apresentaram expressão de pelo menos 1 CTA (sobrevida mediana de 11 meses), a diferença não foi estatisticamente significante (p=0.0947). A resposta humoral espontânea contra pelo menos 1 CTA do painel foi encontrada em 19,6% dos pacientes com LNH. CT47 foi o CTA mais frequentemente expresso (7.2%), seguido do CT45 (5.1%), NY-ESO-1 (5.1%) e MAGE-A4 (5.1%). Os CTAs foram mais frequentemente expressos em LNH de células B (21.2%) comparado aos LNH de células T (6.2%) (p=0.048). Não houve diferença estatisticamente significante na resposta humoral anti-CTAs em qualquer dos outros subgrupos clínicos de LNH. Conclusão: De forma geral, houve baixa expressão protéica de CTAs em nossa casuística de LH e LNH com o painel utilizado. Apesar dos limitados dados disponíveis na literatura, esses achados são concordantes com a maioria dos estudos realizados utilizando RT-PCR e/ou imunohistoquímica. Não houve correlações estatisticamente significantes entre expressão de CTAs e parâmetros clínicos ou prognósticos no presente estudo. A reatividade sérica contra os CTAs testados ocorreu em níveis semelhantes ao da expressão protéica em LNH, sugerindo que os pacientes com LNH são capazes de montar resposta imune humoral específica contra CTAs. / Cancer/testis antigens (CTAs) are expressed in a variety of malignant tumors but in normal adult tissues solely in testicular germ cells. Based on this tumor-associated expression pattern, these antigens are potential targets for immunotherapy. Though carcinomas have been extensively analyzed, less is known about lymphoid malignancies such as lymphomas. Aims: To evaluate the potential of tumor specific antigens as candidates for immunotherapy in lymphomas throughout CTA protein expression and spontaneous humoral immune response analyses. We also aim to investigate clinical correlations and prognostic impact of CTAs expression in lymphomas. Methods: Tissue microarray was generated from 38 Hodgkin´s lymphoma (HL) and 106 non- Hodgkin´s lymphoma (NHL) archival cases. Immunohistochemistry (IHC) was done using a panel of 9 monoclonal antibodies against CTAs. Spontaneous humoral immune response analysis against a larger CTA panel was performed in 97 untreated NHL patient samples, including 59 cases from the TMA cohort, using ELISA technique. Results: We found overall low expression of CTAs in our series of HL (21.1%) and NHL (11.3%) TMAs, being MAGE-A (18.4%) and CT7 (13.2%) the most frequently expressed CTAs in HL, and MAGE-A (6.6%), GAGE (5.7%), NY-ESO-1 (4.8%) and CT7 (4.8%) the most frequently expressed CTAs in NHL. Although we did not find statistically significant difference in CTA expression among the clinicopathological subgroups of HL, CTA positivity was higher in advanced stage (28.6%) compared to early stage patients (11.8%). Among NHL, we found higher CTA expression in aggressive lymphomas (14.9%) compared to indolent lymphomas (3.1%), DLBCL (16.1%) compared to non-DLBCL (6.0%) and in non-complete response group (15.0%) compared to those who achieved complete response (6.8%), but it was not statistically significant. Unexpectedly, early stage disease (19.5%) had higher CTA expression than advanced stage NHL (6.2%). Despite the difference found in survival analysis between NHL patients that presented no CTAs expression (median OS 65 months) and those who expressed at least one CTA (median OS 11 months), it did not reach statistically significant difference (p=0.0947). Serum reactivity against at least 1 CTA was observed in (19.6%) of NHL patients, being more frequent in B-cell lymphomas (21.2%) then T-cell lymphomas (6.2%) (p=0.048). CT47 was the most frequently expressed CTA (7.2%), followed by CT45 (5.1%), NY-ESO-1 (5.1%) and MAGE-A4 (5.1%). Grouping the MAGE-A family similarly to the TMA analysis, we found positivity in 8.2% of NHL serum samples. Among DLBCL, CT45 and NY-ESO-1 were the most frequently expressed CTAs, being positive in 4/50 (8.0%) and 3/50 (6.0%), respectively. Conclusion: We found overall low expression of CTAs in our series of HL and NHL TMAs, and low reactivity against CTA in our serum samples. Our results demonstrated a slightly higher frequency of humoral response against most CTAs included in both TMA and ELISA panel compared to their expression by TMA. Considering that generally a small proportion of patients expressing CTAs develop specific humoral response, it is possible that CTA expression by TMA could be underestimated due to the focal expression pattern in some patients. Therefore, using an extensive panel of antibodies and large TMA and serum cohorts of lymphoma patients, we could not identify CTA candidates for immunotherapy in HL and NHL. / TEDE / BV UNIFESP: Teses e dissertações

CoordenaÃÃo de AperfeiÃoamento de Pessoal de NÃvel Superior / A Leucemia LinfocÃtica Aguda (LLA) e o Linfoma nÃo-Hodgkin (LNH) estÃo entre os mais frequentes tipos de neoplasias em crianÃas. A prevenÃÃo e controle do cÃncer devem ser priorizados, tendo em vista sua alta prevalÃncia e crescente relevÃncia como causa de morte em muitos paÃses, alÃm do grande volume de recursos financeiros consumidos. O nordeste brasileiro à pobre em estudos epidemiolÃgicos sobre o cÃncer infantil. O objetivo deste estudo foi traÃar o perfil dos pacientes portadores de LLA e LNH admitidos no perÃodo de 2001 a 2005 no Hospital Infantil Albert Sabin. Estudo observacional, descritivo e retrospectivo. 325 prontuÃrios (254 casos de LLA e 71 de LNH) de pacientes entre 0 e 18 anos e 11 meses foram revisados. Os dados foram inseridos em bancos de dados dos programas SPSS 14.0, Epi Info 3.3.2 e Microsoft Excel 2007. O ponto de corte para desnutriÃÃo foi o escore Z igual a -2 desvios-padrÃo. Na anÃlise estatÃstica foram utilizados o teste exato de Fisher, Qui-quadrado, Student (t), Mann-Whitney, Shapiro-Wilk, Levene, Log-rank, modelo de regressÃo de Cox e mÃtodo de Kaplan Meier para anÃlise de sobrevida. O nÃvel de significÃncia foi p<0,05. A populaÃÃo deste estudo constituiu-se predominantemente por pacientes do sexo masculino (63,4%), faixa etÃria de 02 a 06 anos (49,8%), cor nÃo-branca (62,5%); provenientes da capital e regiÃo metropolitana (56,9%) e com prognÃstico de alto risco (59,1%). 38,3% evoluÃram a Ãbito. As principais manifestaÃÃes clÃnicas iniciais foram febre, anemia, emagrecimento e cansaÃo nos casos de LLA; e febre, massa tumoral palpÃvel, anemia e dor abdominal nos casos de LNH. O tempo mÃdio de duraÃÃo das queixas foi de 3,9 meses. CrianÃas de 0-1 e de 13-18 anos apresentaram pior prognÃstico. A cor da pele, o prognÃstico, o protocolo de tratamento e os sintomas/sinais iniciais febre, cansaÃo e vÃmito mostraram associaÃÃo significativa em relaÃÃo aos Ãbitos. Os protocolos terapÃuticos mais utilizados foram adaptados do LLA 93 e LNH 95. 31% dos pacientes em uso do LLA 93 e 49,2% em uso do LNH 95 evoluÃram a Ãbito. Maior percentual de Ãbito ocorreu no grupo de alto risco (56,94%) e durante a fase de induÃÃo (36,11%) do protocolo LLA 93. Entre os pacientes de baixo risco, 39,28% faleceram durante a fase de manutenÃÃo e 17,85% apÃs o fim do protocolo. 48,78% dos pacientes de alto risco faleceram durante a fase de induÃÃo. Entre os 25 casos que utilizavam o protocolo LNH 95 e faleceram, 4% correspondiam a Linfoma de alto risco oriundos de cÃlulas T e 96% de cÃlulas B. 53,31% (n=15) dos pacientes com Linfoma de cÃlulas B e risco intermediÃrio para recaÃda faleceram durante o Ciclo A do tratamento. Nove pacientes apresentavam alto risco para recaÃda e 33,34% faleceram na fase de CitorreduÃÃo. O Ãndice de desnutriÃÃo para os pacientes com LLA foi de 8,3%, 6,0% e 5,6% e para LNH foi 12,3%, 14,1% e 15,9% em relaÃÃo a peso/estatura, peso/idade e estatura/idade, respectivamente. Pacientes com LLA apresentaram dÃficit maior no Ãndice peso/estatura, indicativo de um processo de desnutriÃÃo aguda. Maior dÃficit no Ãndice estatura/idade entre os pacientes com LNH indica um processo de desnutriÃÃo crÃnica. Os resultados acerca da frequÃncia dessas patologias, faixa etÃria e sexo foram equivalentes aos encontrados na maioria dos estudos. O prognÃstico inicial e o protocolo terapÃutico indicam uma possÃvel influÃncia sobre o desfecho do tratamento. Estudos adicionais sÃo necessÃrios para avaliar a influÃncia da quimioterapia, cor da pele, estado nutricional e outros fatores sobre o tempo de sobrevida do paciente com cÃncer. Os profissionais de saÃde e a populaÃÃo leiga precisam conhecer melhor e estar atentos Ãs manifestaÃÃes clÃnicas iniciais das neoplasias a fim de facilitar o diagnÃstico precoce. / The Acute Lymphocytic Leukemia (ALL) and the Non-Hodgkin Lymphoma (NHL) are among the most frequent types of cancer in children. The prevention and control of the cancer must be prioritized, in view of its high prevalence and increasing relevance as cause of death in many countries, beyond the great sum of consumed financial resources. The Brazilian northeast is poor in epidemiological studies about cancer in the children. The objective of this paper was to set the profile of the patients with LLA and LNH admitted in the Hospital Infantil Albert Sabin between 2001 and 2005. Observational descriptive and retrospective study. 325 medical registers (254 cases of LLA and 71 of LNH) of patients among 0 and 18 years and 11 months had been revised. The data had been inserted in data bases of the programs SPSS 14.0, Epi 3.3.2 Info and Microsoft Excel 2007. A Z-score cut-off point of <-2 SD was used to classify the malnutrition. The Fisherâs Exact Test, Qui-square, Student (t), Mann-Whitney, Shapiro-Wilk, Levene, Log-rank, Cox regression and Kaplan Meier Survival Probability Estimates were used in the statistical analyses. The level of significance was p<0,05. The population of this study was predominantly male (63.4%), 02 to 06 years age-group (49.8%), non-white (62.5%), from the capital and metropolitan region (56.9%) and with prognostic of high risk (59.1%). 38.3% died. The main clinical manifestations had been fever, anaemia, loss of weight and fatigue in the LLA cases; and fever, anaemia, palpable tumor mass and abdominal pain in the LNH cases. The mean duration time of the complaints was 3.9 months. Children with 0 to 1 and 13 to 18 years had presented worse prognosis. The color of the skin, the prognosis, the treatment protocol and the initial clinical manifestations (fever, fatigue and vomit) had shown significant association in relation to the deaths. The therapeutical protocols more used were adapted of the LLA 93 and LNH 95. 31% of the patients in use of LLA 93 and 49.2% in use of LNH 95 died. The largest percent of deaths were in the group of high risk (56.94%) and in the induction phase (36.11%) of protocol LLA 93. Between the patients of low risk, 39.28% died during the maintenance phase and 17.85% after the end of the protocol. 48.78% of the patients of high risk died during the induction phase. Among the 25 cases that used protocol LNH 95 and died, 4% corresponded to lymphoma of high risk deriving of the cells T and 96% of the cells B. 53.31% (n=15) of the patients with lymphoma of the cells B and intermediate risk to fallen died during the Cycle A of the treatment. Nine patients presented high risk for fallen and 33.34% died in the cytoreduction phase. The malnutrition indices to the LLA patients were of 8.3%, 6.0% and 5.6% and to LNH were 12.3%, 14.1% and 15.9% in relation the weight/height, weight/age and height/age, respectively. Patients with LLA had presented larger deficit in the index weight/height, indicative of a process of acute malnutrition. Larger deficit in the height/age index between the patients with LNH indicates a process of chronic malnutrition. The results about the frequency of these disease, age-group and gender were equivalents to those encountered in the majority of studies. The initial prognosis and the therapeutical protocol indicate an influence on the outcome of the treatment. Other studies are necessary to evaluate the influence of the chemotherapy, color of the skin, nutritional status and other factors on the survival time of the patient with cancer. The professionals of health and the laypeople need to know better and to be intent to the initial clinical manifestations of the neoplasm disease in order to facilitate the precocious diagnosis.

Marginal zone (MZ) lymphomas (MZLs) represent a group of lymphomas originating from B lymphocytes of the “marginal zone” which is the external part of the secondary lymphoid follicles. The WHO classifies MZL into 3 entities; extranodal MZL, splenic MZL and nodal MZL. Extranodal marginal zone lymphoma (EMZL) can arise in different tissues, including the stomach, salivary gland, lung, small bowel, thyroid, ocular adnexa and skin. We present a 25 years old female with a history of angioedema and chronic cutaneous eczema who developed an unusual EMZL. Patient presented with a history of rapidly enlarging skin nodule on her left elbow that had been present for almost one year. Over a period of 2-3 weeks she felt the nodule rapidly changed in size and shape. Excisional biopsy of the mass revealed a lymphoid infiltrate based in the reticular dermis and focally extending into the subcutaneous adipose tissue with formation of disrupted lymphoid follicles positive for CD20, CD23 and BCL2 but negative for CD10, Cyclin D1 and SOX11. Diagnosis was consistent with extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma). Patient on presentation did not have any B symptoms other cutaneous lesions, lymphadenopathy or hepatosplenomegaly. PET scan revealed no evidence of abnormal uptake leading to a final Stage IE definition. Patient initiated definitive radiation therapy. EMZL accounts for 5 -10 % of non-Hodgkin lymphoma. It has been described often in organs that are normally devoid of germinal centers. It may arise in reactive lymphoid tissue induced by chronic inflammation in extranodal sites. Primary cutaneous marginal zone lymphoma (PCMZL) is associated with infectious etiologies such as Borrelia burgdorferi and less commonly with viral infections or in relation to autoimmune disorders. Autoimmune disorders, specifically Sjögren's syndrome is associated with a 30-fold increased risk of marginal zone lymphoma. Localized disease can be treated by local radiotherapy, intralesional injections or excision. Widespread skin disease is usually treated with a CD20 directed monoclonal antibody-Rituximab. Patients with PCMZL usually have an indolent clinical course. Extracutaneous dissemination of MALT Lymphoma is uncommon and happens in 6-8 % of patients. The 5 years overall survival is between 98-100%. Family physicians and dermatologists should have a high index of suspicion for this rare lymphoma subtype especially in patients with inflammatory chronic skin conditions and atopy.