Leucemia Mielóide Crônica em pediatria: perspectivas atuais

Detalhes bibliográficos
Autor(a) principal: Lee, Maria Lúcia de Martino [UNIFESP]
Data de Publicação: 2008
Tipo de documento: Artigo
Idioma: por
Título da fonte: Repositório Institucional da UNIFESP
Texto Completo: http://dx.doi.org/10.1590/S1516-84842008000700014
http://repositorio.unifesp.br/handle/11600/4338
Resumo: Chronic myeloid leukemia (CML) is a rare event in childhood, comprising of less than 5% of all leukemia cases in this age group. CML is characterized by the presence of a specific molecular marker, the Ph+ chromosome, which is responsible for almost all etiopathogenesis, hence, it has clinical and course characteristics that do not differ from the adult population. In pediatrics the therapeutic approach is based mainly on the experience obtained with adult protocols. With bone marrow transplantation (BMT) being the only cure option, this procedure is more effective in patients with compatible related donors and performed during the initial chronic phase of the disease. The great anti-leukemic efficacy seen with imatinib mesylate was responsible for the approval of this drug in pediatric use for intolerant or refractory -interferon treated patients or relapsed patients after BMT. Currently, its use in pediatric patients with recently diagnosed CML, who have a compatible donor, has become a great dilemma. There is no agreement yet on which is the best way to use this drug or even whether it will ever replace BMT. Further studies with longer follow-up periods are still needed.
id UFSP_97e3c03ce7b1bf92beeddf2e08bfaad2
oai_identifier_str oai:repositorio.unifesp.br/:11600/4338
network_acronym_str UFSP
network_name_str Repositório Institucional da UNIFESP
repository_id_str 3465
spelling Leucemia Mielóide Crônica em pediatria: perspectivas atuaisChronic Myeloid Leukemia in pediatrics patients: current approachChronic Myeloid LeukemiapediatricImatinib mesylateLeucemia Mielóide Crônicapediatriamesilato de imatinibeChronic myeloid leukemia (CML) is a rare event in childhood, comprising of less than 5% of all leukemia cases in this age group. CML is characterized by the presence of a specific molecular marker, the Ph+ chromosome, which is responsible for almost all etiopathogenesis, hence, it has clinical and course characteristics that do not differ from the adult population. In pediatrics the therapeutic approach is based mainly on the experience obtained with adult protocols. With bone marrow transplantation (BMT) being the only cure option, this procedure is more effective in patients with compatible related donors and performed during the initial chronic phase of the disease. The great anti-leukemic efficacy seen with imatinib mesylate was responsible for the approval of this drug in pediatric use for intolerant or refractory -interferon treated patients or relapsed patients after BMT. Currently, its use in pediatric patients with recently diagnosed CML, who have a compatible donor, has become a great dilemma. There is no agreement yet on which is the best way to use this drug or even whether it will ever replace BMT. Further studies with longer follow-up periods are still needed.A Leucemia Mielóide Crônica (LMC) constitui evento raro na infância, representando menos de 5% das leucemias nesta faixa etária. Caracteriza-se pela presença de um marcador citogenético específico, cromossomo Ph+, que é responsável por grande parte da etiopatogenia da doença. Possui, portanto, características clínicas e evolutivas que não diferem dos pacientes adultos. Sua abordagem terapêutica em pediatria é baseada principalmente na experiência obtida com os estudos em adultos. Tem no TMO sua única opção de tratamento curativo, sendo este mais efetivo em pacientes com doador aparentado compatível, realizado durante a fase crônica inicial da doença. A grande eficácia antileucêmica observada com o mesilato de imatinibe fez com que a droga fosse aprovada para uso pediátrico em pacientes intolerantes ou refratários ao interferon a, ou recidivados pós-transplante de medula óssea. Seu uso em pacientes pediátricos com LMC de diagnóstico recente, com doador disponível, tornou-se um grande dilema, não existindo até o momento um consenso em relação à melhor forma de se utilizar a droga ou, mesmo, se esta irá em algum momento substituir o TMO. Estudos mais concretos com um seguimento maior ainda necessitam ser realizados.UNIFESP Instituto de Oncologia Pediátrica - GRAACCUNIFESP, Instituto de Oncologia Pediátrica - GRAACCSciELOAssociação Brasileira de Hematologia e Hemoterapia e Terapia CelularUniversidade Federal de São Paulo (UNIFESP)Lee, Maria Lúcia de Martino [UNIFESP]2015-06-14T13:38:30Z2015-06-14T13:38:30Z2008-04-01info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersion59-65application/pdfhttp://dx.doi.org/10.1590/S1516-84842008000700014Revista Brasileira de Hematologia e Hemoterapia. Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular, v. 30, p. 59-65, 2008.10.1590/S1516-84842008000700014S1516-84842008000700014.pdf1516-8484S1516-84842008000700014http://repositorio.unifesp.br/handle/11600/4338porRevista Brasileira de Hematologia e Hemoterapiainfo:eu-repo/semantics/openAccessreponame:Repositório Institucional da UNIFESPinstname:Universidade Federal de São Paulo (UNIFESP)instacron:UNIFESP2024-08-06T08:19:29Zoai:repositorio.unifesp.br/:11600/4338Repositório InstitucionalPUBhttp://www.repositorio.unifesp.br/oai/requestbiblioteca.csp@unifesp.bropendoar:34652024-08-06T08:19:29Repositório Institucional da UNIFESP - Universidade Federal de São Paulo (UNIFESP)false
dc.title.none.fl_str_mv Leucemia Mielóide Crônica em pediatria: perspectivas atuais
Chronic Myeloid Leukemia in pediatrics patients: current approach
title Leucemia Mielóide Crônica em pediatria: perspectivas atuais
spellingShingle Leucemia Mielóide Crônica em pediatria: perspectivas atuais
Lee, Maria Lúcia de Martino [UNIFESP]
Chronic Myeloid Leukemia
pediatric
Imatinib mesylate
Leucemia Mielóide Crônica
pediatria
mesilato de imatinibe
title_short Leucemia Mielóide Crônica em pediatria: perspectivas atuais
title_full Leucemia Mielóide Crônica em pediatria: perspectivas atuais
title_fullStr Leucemia Mielóide Crônica em pediatria: perspectivas atuais
title_full_unstemmed Leucemia Mielóide Crônica em pediatria: perspectivas atuais
title_sort Leucemia Mielóide Crônica em pediatria: perspectivas atuais
author Lee, Maria Lúcia de Martino [UNIFESP]
author_facet Lee, Maria Lúcia de Martino [UNIFESP]
author_role author
dc.contributor.none.fl_str_mv Universidade Federal de São Paulo (UNIFESP)
dc.contributor.author.fl_str_mv Lee, Maria Lúcia de Martino [UNIFESP]
dc.subject.por.fl_str_mv Chronic Myeloid Leukemia
pediatric
Imatinib mesylate
Leucemia Mielóide Crônica
pediatria
mesilato de imatinibe
topic Chronic Myeloid Leukemia
pediatric
Imatinib mesylate
Leucemia Mielóide Crônica
pediatria
mesilato de imatinibe
description Chronic myeloid leukemia (CML) is a rare event in childhood, comprising of less than 5% of all leukemia cases in this age group. CML is characterized by the presence of a specific molecular marker, the Ph+ chromosome, which is responsible for almost all etiopathogenesis, hence, it has clinical and course characteristics that do not differ from the adult population. In pediatrics the therapeutic approach is based mainly on the experience obtained with adult protocols. With bone marrow transplantation (BMT) being the only cure option, this procedure is more effective in patients with compatible related donors and performed during the initial chronic phase of the disease. The great anti-leukemic efficacy seen with imatinib mesylate was responsible for the approval of this drug in pediatric use for intolerant or refractory -interferon treated patients or relapsed patients after BMT. Currently, its use in pediatric patients with recently diagnosed CML, who have a compatible donor, has become a great dilemma. There is no agreement yet on which is the best way to use this drug or even whether it will ever replace BMT. Further studies with longer follow-up periods are still needed.
publishDate 2008
dc.date.none.fl_str_mv 2008-04-01
2015-06-14T13:38:30Z
2015-06-14T13:38:30Z
dc.type.driver.fl_str_mv info:eu-repo/semantics/article
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
format article
status_str publishedVersion
dc.identifier.uri.fl_str_mv http://dx.doi.org/10.1590/S1516-84842008000700014
Revista Brasileira de Hematologia e Hemoterapia. Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular, v. 30, p. 59-65, 2008.
10.1590/S1516-84842008000700014
S1516-84842008000700014.pdf
1516-8484
S1516-84842008000700014
http://repositorio.unifesp.br/handle/11600/4338
url http://dx.doi.org/10.1590/S1516-84842008000700014
http://repositorio.unifesp.br/handle/11600/4338
identifier_str_mv Revista Brasileira de Hematologia e Hemoterapia. Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular, v. 30, p. 59-65, 2008.
10.1590/S1516-84842008000700014
S1516-84842008000700014.pdf
1516-8484
S1516-84842008000700014
dc.language.iso.fl_str_mv por
language por
dc.relation.none.fl_str_mv Revista Brasileira de Hematologia e Hemoterapia
dc.rights.driver.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv 59-65
application/pdf
dc.publisher.none.fl_str_mv Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular
publisher.none.fl_str_mv Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular
dc.source.none.fl_str_mv reponame:Repositório Institucional da UNIFESP
instname:Universidade Federal de São Paulo (UNIFESP)
instacron:UNIFESP
instname_str Universidade Federal de São Paulo (UNIFESP)
instacron_str UNIFESP
institution UNIFESP
reponame_str Repositório Institucional da UNIFESP
collection Repositório Institucional da UNIFESP
repository.name.fl_str_mv Repositório Institucional da UNIFESP - Universidade Federal de São Paulo (UNIFESP)
repository.mail.fl_str_mv biblioteca.csp@unifesp.br
_version_ 1814268295758479360