Publicação
Mucosa-associated lymphoid tissue lymphomas
| Resumo: | Mucosa-associated lymphoid tissue (MALT) lymphoma is an indolent extranodal marginal zone B-cell lymphoma, originating in acquired MALT that is induced in mucosal barriers as part of a normal adaptive immune response to a chronic immunoinflammatory stimulus, including infections by Helicobacter pylori, Borrelia burgdorferi and Chlamydophila psittaci and autoimmune diseases. This antigenic stimulation initially leads to lymphoid hyperplasia; the acquisition of additional genetic aberrations culminates in the activation of intracellular survival pathways, with disease progression due to proliferation and resistance to apoptosis. While early-stage disease can frequently regress through the therapeutic reversal of the chronic immune stimulus, the presence of immortalizing genetic abnormalities or of advanced disease requires a more aggressive approach which is, presently, not consensual. This lymphoma is a rare neoplasm, with a worldwide incidence of 1-1.5 cases per 105, per year. There are descriptions of MALT lymphomas affecting practically every organ and system, with a marked geographic variability partially attributable to the epidemiology of the underlying risk factors. The rarer locations, representing less than 1% of all cases, can have yearly incidences as low as 1 per 108, determining an inability to accrue representative series of patients for epidemiologic studies and robust clinical trials that could sustain informed evidence-based therapeutic decisions. The present review article aims to update the state of the art of knowledge regarding the ethiopathogenesis and oncobiology of this rare malignancy, while summarizing the latest clinical results, to improve the evidence for a clinical decision and optimize the quality of patient care. |
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| Autores principais: | Pereira, Marta Isabel de Correia |
| Assunto: | Linfoma de tecido linfóide associado a mucosa Helicobacter pylori Mucosa |
| Ano: | 2012 |
| País: | Portugal |
| Tipo de documento: | dissertação de mestrado |
| Tipo de acesso: | acesso aberto |
| Instituição associada: | Universidade de Coimbra |
| Idioma: | inglês |
| Origem: | Estudo Geral - Universidade de Coimbra |
| Resumo: | Mucosa-associated lymphoid tissue (MALT) lymphoma is an indolent extranodal marginal zone B-cell lymphoma, originating in acquired MALT that is induced in mucosal barriers as part of a normal adaptive immune response to a chronic immunoinflammatory stimulus, including infections by Helicobacter pylori, Borrelia burgdorferi and Chlamydophila psittaci and autoimmune diseases. This antigenic stimulation initially leads to lymphoid hyperplasia; the acquisition of additional genetic aberrations culminates in the activation of intracellular survival pathways, with disease progression due to proliferation and resistance to apoptosis. While early-stage disease can frequently regress through the therapeutic reversal of the chronic immune stimulus, the presence of immortalizing genetic abnormalities or of advanced disease requires a more aggressive approach which is, presently, not consensual. This lymphoma is a rare neoplasm, with a worldwide incidence of 1-1.5 cases per 105, per year. There are descriptions of MALT lymphomas affecting practically every organ and system, with a marked geographic variability partially attributable to the epidemiology of the underlying risk factors. The rarer locations, representing less than 1% of all cases, can have yearly incidences as low as 1 per 108, determining an inability to accrue representative series of patients for epidemiologic studies and robust clinical trials that could sustain informed evidence-based therapeutic decisions. The present review article aims to update the state of the art of knowledge regarding the ethiopathogenesis and oncobiology of this rare malignancy, while summarizing the latest clinical results, to improve the evidence for a clinical decision and optimize the quality of patient care. |
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