OBJECTIVES: The aim of this retrospective study was to investigate the results of T-cell large granular lymphocytic leukemia treatment with fludarabine by assessing the complete hematologic response, the complete molecular response, progression-free survival, and overall survival. METHODS: We evaluated the records of six patients with T-cell large granular lymphocytic leukemia who were treated with fludarabine as a first-, second-, or third-line therapy, at a dose of 40 mg/m², for three to five days per month and 6 to 8 cycles. RESULTS: Of the six patients investigated with T-cell large granular lymphocytic leukemia who were treated with fludarabine, five (83.3%) were female, and their median age was 36.5 years (range 18 to 73). The median lymphocyte level was 3.4x10(9)/L (0.5 to 8.9). All patients exhibited a monoclonal T-cell receptor gamma gene rearrangement at diagnosis. Two (33.3%) patients received fludarabine as first-line treatment, two (33.3%) for refractory disease, one (16.6%) for relapsed disease after the suspension of methotrexate treatment dueto liver toxicity, and one (16.6%) due to dyspesia. A complete hematologic response was achieved in all cases, and a complete molecular response was achieved in five out six cases (83.3%). During a mean follow-up period of 12 months, both the progression-free survival and overall survival rates were 100%. CONCLUSION: T-cell large granular lymphocytic leukemia demonstrated a high rate of complete hematologic and molecular response to fludarabine, with excellent compliance and tolerability rates. To confirm our results in this rare disease, we believe that fludarabine should be tested in clinical trials as a first-line treatment for T-cell large granular lymphocytic leukemia.

INTRODUÇÃO: A classificação da Organização Mundial da Saúde (OMS) para os tumores do tecido hematopoético e linfoide (4ª edição, 2008) representa uma revisão atualização da 3ª edição publicada em 2001. A tradução da nomenclatura utilizada para identificar as entidades descritas deve ser clara, precisa e uniforme no sentido de reproduzir de forma correta as diversas entidades clinicopatológicas para clínicos, patologistas e pesquisadores envolvidos na área da onco-hematopatologia. OBJETIVO: Os autores apresentam uma proposta de atualização e padronização terminológica em língua portuguesa, com base na OMS/2008

INTRODUCTION: The World Health Organization (WHO) classification of hematopoietic and lymphoid tissue (4th edition, 2008) tumors constitutes an updated review of the 3rd edition published in 2001. The translation of the nomenclature used to describe the entities should be clear, precise and uniform so that clinicians, pathologists and researchers involved in the onco-hematopathological area may identify them accurately. OBJECTIVE: With this purpose, the authors present an updated proposal and a terminological standardization in Portuguese based on WHO/2008

Os linfomas extralinfonodais representam aproximadamente 1/3 de todos os linfomas não Hodgkin (LNH) e, embora possam ter início em qualquer tecido, mais frequentemente acometem o trato gastrointestinal, sendo o estômago o órgão responsável pela grande maioria dos casos. Os linfomas primários gástricos são comumente LNH, sendo representados em mais de 95% dos casos pelo linfoma difuso de grandes células B e pelo linfoma MALT (mucosa associated lymphoid tissue). De evolução indolente, o linfoma MALT destaca-se por ser um modelo de câncer secundário à estimulação antigênica crônica exercida por uma bactéria denominada Helicobacter pylori (HP). No outro polo, situa-se o linfoma difuso de células B (LDGCB), que, de patogênese duvidosa, pode tratar-se de uma transformação de LNH MALT ou ainda se caracterizar por um linfoma "de novo". Neste estudo, revisamos a literatura, enfatizando aspectos importantes à prática clínica destes linfomas.

Extranodal lymphomas account for about 30% of all non-Hodgkin lymphomas (NHL), and although they can originate in any tissue, the gastrointestinal tract is the most commonly affected structure with the stomach being the most common subtype. Diffuse Large B cell lymphoma (DLBCL) and MALT (mucosa associated lymphoid tissue) lymphoma account for more than 95% of the cases of gastric lymphoma. The indolent development of MALT lymphoma stands out as it is a type of cancer subject to chronic antigen stimulation by the Helicobacter pylori bacteria. Conversely, diffuse large B cell lymphomas, whose pathogenesis is uncertain, can be a transformation from MALT NHL or perhaps a new type of lymphoma. In this study we carried out a review of the literature, stressing the key aspects of these lymphomas in the clinical practice.

Estudo retrospectivo que visa analisar a utilidade da biópsia de medula óssea (BMO) bilateral na infiltração de medula óssea (MO) por linfoma difuso de grandes células B (LDGCB). Nossos objetivos foram avaliar a incidência de infiltração unilateral de MO por LDGCB e comparar o comprimento dos fragmentos obtidos entre as amostras positivas e negativas para infiltração. Além disso, verificamos se houve diferença entre os casos com infiltração unilateral versus bilateral, correlacionando com desidrogenase láctica (DHL) e estadiamento tomográfico. Avaliamos 268 casos de LDGCB e observamos infiltração medular em 34 casos (13%). Não foi possível a avaliação de seis casos, restando 28 casos para análise. Foram revisados no total 70 fragmentos de MO sobre presença ou ausência de infiltração e comprimento. A média do número de fragmentos por casos foi 2,5; a média do comprimento dos fragmentos foi 11,01 mm (± 5,12 mm), e a média do comprimento dos fragmentos por caso foi 27,53 mm. Foi observado que em seis casos (21,4%) havia infiltração unilateral. Não foram evidenciadas diferenças nas médias do comprimento dos fragmentos em relação à presença versus ausência de infiltração 10,95 mm (± 5,1 mm) versus 11,57 mm (± 5,2 mm), p > 0,05, respectivamente. Não foram evidenciadas diferenças em 23 casos entre a comparação da infiltração medular unilateral versus bilateral com DHL e estadiamento tomográfico. Concluímos que a BMO bilateral foi superior à unilateral, pois pode aumentar a detecção de infiltração de MO em 21,4% dos casos.

This retrospective study aims to analyze the usefulness of bilateral bone marrow biopsy in bone marrow infiltration by diffuse large B-cell lymphoma (DLBCL). Our objectives were to assess the incidence of unilateral BM involvement by DLBCL and compare fragment length obtained from positive and negative samples for infiltration. Furthermore, we compared the differences between unilateral and bilateral infiltration correlating with lactic dehydrogenase (LDH) and computerized tomography (CT) staging. We evaluated 268 cases of DLBCL and observed medullary infiltration in 34 cases (13%). It was not possible to evaluate 6 out of 34 cases. 70 BM fragments were reviewed as to the presence or absence of infiltration and length. The mean number of fragments per case was 2.5; the mean BM fragment length was 11.01 mm (± 5.12 mm) and the mean BM fragment length per case was 27.53 mm. There was unilateral BM infiltration in six cases (21.4%). There were no differences in the mean fragment length as to the presence/absence of infiltration 10.95 mm (± 5.2 mm) versus 11.57 mm, p > 0.05, respectively. There were no differences in 23 cases between the comparison of unilateral medullary infiltration versus bilateral with lactic dehydrogenase and CT staging. We concluded that bilateral bone marrow biopsy was superior to unilateral because it may increase by 21.4% the detection of BM involvement by DLBCL.

CONTEXTO E OBJETIVO: Tem-se afirmado correntemente que, em países industrializados, a esclerose nodular é o tipo mais freqüente de linfoma de Hodgkin, ao contrário de países em desenvolvimento, onde a celularidade mista e a depleção linfocitária são mais freqüentes. O objetivo do estudo é rever os dados histológicos de linfoma de Hodgkin das cidades de São Paulo e Campinas. TIPO DE ESTUDO E LOCAL: Transversal, por análise histopatológica, em quatro hospitais universitários e um centro oncológico de referência. MÉTODOS: 1.025 casos com o diagnóstico de linfoma de Hodgkin entre 1990 e 2000 foram coletados de cinco instituições. Em 631 (61,5%) casos fora feito estudo imunoistoquímico para os marcadores CD20, CD3, CD15 e CD30. As freqüências relativas dos tipos histológicos (informadas pelos autores que são hematopatologistas de suas respectivas instituições) foram determinadas nos diversos grupos etários e por gênero. RESULTADOS: Os tipos de linfoma de Hodgkin foram assim distribuídos: predominância linfocitária 4,8%, esclerose nodular 69,2%, celularidade mista 21,1% e depleção linfocitária 4,6%. CONCLUSÕES: Dados controversos sobre a freqüência dos tipos de linfoma de Hodgkin em nosso meio parecem ser devidos ao pequeno número de casos dos trabalhos anteriores. Nossos dados são comparáveis aos dos países industrializados.

CONTEXT AND OBJECTIVE: It is currently asserted that, in industrialized countries, nodular sclerosis is the most frequent type of Hodgkin's lymphoma, in contrast to developing countries, where mixed cellularity and lymphocyte depletion are more frequently seen. The objective was to review histological data from cases of Hodgkin's lymphoma from São Paulo and Campinas cities. DESIGN AND SETTING: Cross-sectional histopathological analysis, in four university hospitals and one cancer care center. METHODS: 1,025 cases diagnosed as Hodgkin's lymphoma between 1990 and 2000 were collected from five institutions; 631 of them (61.5%) had been immunophenotyped using antibodies to CD20, CD3, CD15 and CD30. The relative frequencies of histological types (as informed by the contributing authors, who are hematopathologists in their institutions) were determined according to age and gender. RESULTS: The Hodgkin's lymphoma types were distributed as follows: lymphocyte predominance 4.8%, nodular sclerosis 69.2%, mixed cellularity 21.1% and lymphocyte depletion 4.6%. CONCLUSIONS: The controversy regarding the frequencies of Hodgkin's lymphoma types within the Brazilian setting seems to be due to the small number of cases in previous studies. The present data show a picture close to the situation in the industrialized countries.