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Biology and Pathogenesis of B Non-Hodgkin Lymphoma in Childhood: a Review

Bibliographic Details
Main Author: Klumb, Claudete Esteves
Publication Date: 2001
Format: Article
Language: por
Source: Revista Brasileira de Cancerologia (Online)
DOI: 10.32635/2176-9745.RBC.2001v47n3.2308
Download full: https://rbc.inca.gov.br/index.php/revista/article/view/2308
Summary: The non-Hodgkin’s lymphoma in childhood encompasses a closely related group of aggressive Bcell tumors that includes Burkitt’s and non Burkitt’s lymphomas among other less frequent types. Burkitt’s lymphoma includes two distinct forms, namely the endemic and sporadic types, which are characterized by the presence of a chromosomal translocation leading to c-myc proto-oncogene deregulation. Although the normal function of c-myc remains enigmatic, recent data indicate that it has a central role in several fundamental aspects of cellular biology, including proliferation, differentiation, metabolism, and apoptosis. This review focuses on the clinical, epidemiological and molecular features of B non-Hodgkin’s in childhood and discusses new insights into molecular mechanisms of c-myc activation associated to p53 and Rb gene mutations with Burkitt’s lymphoma pathogenesis. The role of EBV virus infection and its potential relevance to virus-induced carcinogenesis and the interaction of viral factors with cellular tumor supressor proteins is also discussed.
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spelling Biology and Pathogenesis of B Non-Hodgkin Lymphoma in Childhood: a Review Biologia e Patogênese dos Linfomas não Hodgkin de Origem B na Infância: uma Revisão Linfoma não HodgkinLinfoma de BurkittCriançaNon-Hodgkin’s LymphomaBurkitt’s LymphomaChildThe non-Hodgkin’s lymphoma in childhood encompasses a closely related group of aggressive Bcell tumors that includes Burkitt’s and non Burkitt’s lymphomas among other less frequent types. Burkitt’s lymphoma includes two distinct forms, namely the endemic and sporadic types, which are characterized by the presence of a chromosomal translocation leading to c-myc proto-oncogene deregulation. Although the normal function of c-myc remains enigmatic, recent data indicate that it has a central role in several fundamental aspects of cellular biology, including proliferation, differentiation, metabolism, and apoptosis. This review focuses on the clinical, epidemiological and molecular features of B non-Hodgkin’s in childhood and discusses new insights into molecular mechanisms of c-myc activation associated to p53 and Rb gene mutations with Burkitt’s lymphoma pathogenesis. The role of EBV virus infection and its potential relevance to virus-induced carcinogenesis and the interaction of viral factors with cellular tumor supressor proteins is also discussed.Os linfomas não Hodgkin de origem B na infância são neoplasias de alto grau de malignidade, predominando os linfomas do tipo Burkitt e não Burkitt. O linfoma de Burkitt apresenta dois subtipos, o endêmico e o esporádico, ambos caracterizados por translocações cromossômicas envolvendo o proto-oncogene c-myc que resultam na perda de regulação desse gene. Embora a função normal do c-myc permaneça enigmática, recentes dados indicam que esse gene desempenha um papel importante em diversos aspectos da biologia celular, incluindo proliferação, diferenciação, metabolismo e apoptose. Nesta revisão são abordados os aspectos epidemiológicos, clínicos e moleculares dos linfomas B da infância e o papel da ativação do gene c-myc associado a mutações dos genes p53 e Rb na patogênese dos linfomas de Burkitt. O papel da infecção pelo vírus Epstein-Barr (EBV), sua relevância na indução da carcinogenese e interação das proteínas virais com proteínas dos genes supressores de tumor é também discutido.INCA2001-09-28info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionArtigos, Avaliado pelos paresapplication/pdfhttps://rbc.inca.gov.br/index.php/revista/article/view/230810.32635/2176-9745.RBC.2001v47n3.2308Revista Brasileira de Cancerologia; Vol. 47 No. 3 (2001): July/Aug./Sept.; 291-301Revista Brasileira de Cancerologia; Vol. 47 Núm. 3 (2001): jul./ago./sept.; 291-301Revista Brasileira de Cancerologia; v. 47 n. 3 (2001): jul./ago/set.; 291-3012176-9745reponame:Revista Brasileira de Cancerologia (Online)instname:Instituto Nacional de Câncer José Alencar Gomes da Silva (INCA)instacron:INCAporhttps://rbc.inca.gov.br/index.php/revista/article/view/2308/1441Klumb, Claudete Esteves info:eu-repo/semantics/openAccess2021-11-29T20:41:07Zoai:rbc.inca.gov.br:article/2308Revistahttps://rbc.inca.gov.br/index.php/revistaPUBhttps://rbc.inca.gov.br/index.php/revista/oairbc@inca.gov.br0034-71162176-9745opendoar:2021-11-29T20:41:07Revista Brasileira de Cancerologia (Online) - Instituto Nacional de Câncer José Alencar Gomes da Silva (INCA)false
dc.title.none.fl_str_mv Biology and Pathogenesis of B Non-Hodgkin Lymphoma in Childhood: a Review
Biologia e Patogênese dos Linfomas não Hodgkin de Origem B na Infância: uma Revisão
title Biology and Pathogenesis of B Non-Hodgkin Lymphoma in Childhood: a Review
spellingShingle Biology and Pathogenesis of B Non-Hodgkin Lymphoma in Childhood: a Review
Biology and Pathogenesis of B Non-Hodgkin Lymphoma in Childhood: a Review
Klumb, Claudete Esteves
Linfoma não Hodgkin
Linfoma de Burkitt
Criança
Non-Hodgkin’s Lymphoma
Burkitt’s Lymphoma
Child
Klumb, Claudete Esteves
Linfoma não Hodgkin
Linfoma de Burkitt
Criança
Non-Hodgkin’s Lymphoma
Burkitt’s Lymphoma
Child
title_short Biology and Pathogenesis of B Non-Hodgkin Lymphoma in Childhood: a Review
title_full Biology and Pathogenesis of B Non-Hodgkin Lymphoma in Childhood: a Review
title_fullStr Biology and Pathogenesis of B Non-Hodgkin Lymphoma in Childhood: a Review
Biology and Pathogenesis of B Non-Hodgkin Lymphoma in Childhood: a Review
title_full_unstemmed Biology and Pathogenesis of B Non-Hodgkin Lymphoma in Childhood: a Review
Biology and Pathogenesis of B Non-Hodgkin Lymphoma in Childhood: a Review
title_sort Biology and Pathogenesis of B Non-Hodgkin Lymphoma in Childhood: a Review
author Klumb, Claudete Esteves
author_facet Klumb, Claudete Esteves
Klumb, Claudete Esteves
author_role author
dc.contributor.author.fl_str_mv Klumb, Claudete Esteves
dc.subject.por.fl_str_mv Linfoma não Hodgkin
Linfoma de Burkitt
Criança
Non-Hodgkin’s Lymphoma
Burkitt’s Lymphoma
Child
topic Linfoma não Hodgkin
Linfoma de Burkitt
Criança
Non-Hodgkin’s Lymphoma
Burkitt’s Lymphoma
Child
description The non-Hodgkin’s lymphoma in childhood encompasses a closely related group of aggressive Bcell tumors that includes Burkitt’s and non Burkitt’s lymphomas among other less frequent types. Burkitt’s lymphoma includes two distinct forms, namely the endemic and sporadic types, which are characterized by the presence of a chromosomal translocation leading to c-myc proto-oncogene deregulation. Although the normal function of c-myc remains enigmatic, recent data indicate that it has a central role in several fundamental aspects of cellular biology, including proliferation, differentiation, metabolism, and apoptosis. This review focuses on the clinical, epidemiological and molecular features of B non-Hodgkin’s in childhood and discusses new insights into molecular mechanisms of c-myc activation associated to p53 and Rb gene mutations with Burkitt’s lymphoma pathogenesis. The role of EBV virus infection and its potential relevance to virus-induced carcinogenesis and the interaction of viral factors with cellular tumor supressor proteins is also discussed.
publishDate 2001
dc.date.none.fl_str_mv 2001-09-28
dc.type.driver.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
Artigos, Avaliado pelos pares
format article
status_str publishedVersion
dc.identifier.uri.fl_str_mv https://rbc.inca.gov.br/index.php/revista/article/view/2308
10.32635/2176-9745.RBC.2001v47n3.2308
url https://rbc.inca.gov.br/index.php/revista/article/view/2308
identifier_str_mv 10.32635/2176-9745.RBC.2001v47n3.2308
dc.language.iso.fl_str_mv por
language por
dc.relation.none.fl_str_mv https://rbc.inca.gov.br/index.php/revista/article/view/2308/1441
dc.rights.driver.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.publisher.none.fl_str_mv INCA
publisher.none.fl_str_mv INCA
dc.source.none.fl_str_mv Revista Brasileira de Cancerologia; Vol. 47 No. 3 (2001): July/Aug./Sept.; 291-301
Revista Brasileira de Cancerologia; Vol. 47 Núm. 3 (2001): jul./ago./sept.; 291-301
Revista Brasileira de Cancerologia; v. 47 n. 3 (2001): jul./ago/set.; 291-301
2176-9745
reponame:Revista Brasileira de Cancerologia (Online)
instname:Instituto Nacional de Câncer José Alencar Gomes da Silva (INCA)
instacron:INCA
instname_str Instituto Nacional de Câncer José Alencar Gomes da Silva (INCA)
instacron_str INCA
institution INCA
reponame_str Revista Brasileira de Cancerologia (Online)
collection Revista Brasileira de Cancerologia (Online)
repository.name.fl_str_mv Revista Brasileira de Cancerologia (Online) - Instituto Nacional de Câncer José Alencar Gomes da Silva (INCA)
repository.mail.fl_str_mv rbc@inca.gov.br
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dc.identifier.doi.none.fl_str_mv 10.32635/2176-9745.RBC.2001v47n3.2308