Leucemia Mielóide Crônica em pediatria: perspectivas atuais
Autor(a) principal: | |
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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. |
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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 |
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1814268295758479360 |