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      Processos linfoproliferativos da pele: Parte 1 - Linfomas cutâneos de células B Translated title: Lymphoproliferative processes of the skin: Part 1 - Primary cutaneous B-cell lymphomas

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          Abstract

          Os linfomas cutâneos primários de células B pertencem ao grupo das neoplasias malignas originadas de linfócitos B, do tipo não-Hodgkin. A rotina diagnóstica nos processos linfoproliferativos de células B é realizada pela biópsia da pele lesada para a análise histopatológica, imuno-histoquímica e pesquisa do rearranjo gênico. A classificação dos linfomas cutâneos primários vem sendo discutida nos últimos anos; as usualmente utilizadas são as propostas pela World Health Organization - WHO e pela European Organization for Research and Treatment of Cancer - EORTC. A recente classificação consensual proposta por WHO-EORTC deverá substituí-las. Entretanto, apesar dos recentes progressos, ainda existem controvérsias e dificuldades quanto à classificação, ao diagnóstico e ao tratamento dos linfomas cutâneos primários de células B.

          Translated abstract

          Primary cutaneous B-cell lymphomas comprise a group of malignant neoplasms originated from the B-cell lymphoid lineage, of the non-Hodgkin type. The diagnostic routine of cutaneous B-cell lymphoproliferative processes consists of a skin biopsy for histopathological, immunohistochemical and gene rearrangement analysis. The classification of primary cutaneous lymphomas is still controversial. The currently most usual classifications are those proposed by the World Health Organization (WHO) and by the European Organization for Research and Treatment of Cancer (EORTC). The recently proposed WHO-EORTC consensus classification should replace them. However, despite recent progress in this field, there are still controversies and difficulties, mainly regarding the diagnosis and classification of cutaneous B-cell lymphomas.

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          Most cited references62

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          Absence of the t(14;18) chromosomal translocation in primary cutaneous B-cell lymphoma.

          The t(14;18)(q32;q21) chromosomal translocation is found in the majority of nodal follicular lymphomas and in a lower percentage of systemic high-grade diffuse large B-cell lymphomas. The translocation results in the juxtaposition of the bcl-2 gene on chromosome 18 with the immunoglobulin heavy chain joining region on chromosome 14. Bcl-2 protein prevents apoptosis and the translocation leads to overexpression of a functionally normal Bcl-2 protein that prevents apoptosis of neoplastic cells. The purpose of our study was to analyse cases of primary cutaneous B-cell lymphoma (PCBCL) for the presence of the t(14;18) translocation and to correlate the results with Bcl-2 expression and histological subtype. Forty-four cutaneous B-cell lymphoid proliferations (36 PCBCL, four follicular B-cell lymphomas with cutaneous presentation and four reactive B-cell infiltrates) were analysed by polymerase chain reaction amplification and polyacrylamide gel electrophoresis using consensus primers for the joining region on the immunoglobulin heavy chain gene in combination with either a primer for the major breakpoint region (MBR) or the minor cluster region (mcr) on chromosome 18. None of 36 PCBCL analysed demonstrated a t(14;18) translocation; however, three of four systemic follicular B-cell lymphomas presenting in the skin were found to have a translocation in the MBR, which was confirmed by sequence analysis. Correlation with Bcl-2 immunostaining showed that of seven patients with high-grade cutaneous diffuse large B-cell lymphoma, four were Bcl-2 positive but had no evidence of a t(14;18) translocation. In the five cases classified as primary cutaneous follicle centre cell lymphoma, the neoplastic cells within the germinal centres failed to express Bcl-2. However, Bcl-2-positive neoplastic cells were present in all four cases of systemic follicular lymphoma, including the case that did not show a t(14;18) translocation. In all cases of marginal zone lymphoma the marginal zone lymphocytes were Bcl-2 positive. These findings indicate that the t(14;18) translocation does not occur in PCBCL, which suggests the involvement of different pathogenetic mechanisms compared with their nodal counterparts. Furthermore, the detection of a t(14;18) translocation in cutaneous B-cell lymphoma should suggest the presence of systemic disease, which underlies the need for exhaustive staging procedures.
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            The monoclonality of human B-cell lymphomas

            R. Levy (1977)
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              Antigen-dependent B-cell development.

              There is growing evidence that the development of naïve B cells depends on the interaction of self antigens with the BCR. A view that has emerged over the past year is that BCR signal output contributes in a large part to the developmental fate of peripheral B cells. Differences in antigen-receptor signal strength may determine whether B cells assume a marginal zone, follicular or B-1 phenotype.
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                Author and article information

                Contributors
                Role: ND
                Role: ND
                Journal
                abd
                Anais Brasileiros de Dermatologia
                An. Bras. Dermatol.
                Sociedade Brasileira de Dermatologia (Rio de Janeiro )
                1806-4841
                October 2005
                : 80
                : 5
                : 461-471
                Affiliations
                [1 ] Universidade de São Paulo Brazil
                Article
                S0365-05962005000600003
                10.1590/S0365-05962005000600003
                44802fa2-c6a3-43f6-89a7-ed34a2ca52ec

                http://creativecommons.org/licenses/by/4.0/

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                Product

                SciELO Brazil

                Self URI (journal page): http://www.scielo.br/scielo.php?script=sci_serial&pid=0365-0596&lng=en
                Categories
                DERMATOLOGY

                Dermatology
                B-lymphocytes,Lymphoma, B-cell,Lymphoma, non-Hodgkin,Skin neoplasms,Lymphoproliferative disorders,Linfócitos B,Linfoma de células B,Linfoma não Hodgkin,Neoplasias cutâneas,Transtornos linfoproliferativos

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