Essential thrombocythemia: a rare disease in childhood

Detalhes bibliográficos
Autor(a) principal: Beatrice,Julia Maimone
Data de Publicação: 2013
Outros Autores: Garanito,Marlene Pereira
Tipo de documento: Relatório
Idioma: eng
Título da fonte: Revista brasileira de hematologia e hemoterapia (Online)
Texto Completo: http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842013000400287
Resumo: Essential thrombocythemia is an acquired myeloproliferative disorder characterized by the proliferation of megakaryocytes in bone marrow, leading to a persistent increase in the number of circulating platelets and thus increasing the risk for thrombotic and hemorrhagic events. The disease features leukocytosis, splenomegaly, vascular occlusive events, hemorrhages and vasomotor disorders. The intricate mechanisms underlying the molecular pathogenesis of this disorder are not completely understood and are still a matter of discussion. Essential thrombocythemia is an extremely rare disorder during childhood. We report on a case of essential thrombocythemia in a child and discuss the diagnostic approach and treatment strategy.
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spelling Essential thrombocythemia: a rare disease in childhoodThrombocythemia, essential/drug therapyThrombocytosisThrombosisMyelodysplastic-myeloproliferative diseasesPlatelet aggregation inhibitors/therapeutic useHydroxyurea/therapeutic useHumansCase reportsFemaleChildEssential thrombocythemia is an acquired myeloproliferative disorder characterized by the proliferation of megakaryocytes in bone marrow, leading to a persistent increase in the number of circulating platelets and thus increasing the risk for thrombotic and hemorrhagic events. The disease features leukocytosis, splenomegaly, vascular occlusive events, hemorrhages and vasomotor disorders. The intricate mechanisms underlying the molecular pathogenesis of this disorder are not completely understood and are still a matter of discussion. Essential thrombocythemia is an extremely rare disorder during childhood. We report on a case of essential thrombocythemia in a child and discuss the diagnostic approach and treatment strategy.Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular2013-01-01info:eu-repo/semantics/reportinfo:eu-repo/semantics/publishedVersiontext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842013000400287Revista Brasileira de Hematologia e Hemoterapia v.35 n.4 2013reponame:Revista brasileira de hematologia e hemoterapia (Online)instname:Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular (ABHHTC)instacron:ABHHTC10.5581/1516-8484.20130059info:eu-repo/semantics/openAccessBeatrice,Julia MaimoneGaranito,Marlene Pereiraeng2013-10-10T00:00:00Zoai:scielo:S1516-84842013000400287Revistahttp://www.rbhh.org/pt/archivo/https://old.scielo.br/oai/scielo-oai.phpsbhh@terra.com.br||secretaria@rbhh.org1806-08701516-8484opendoar:2013-10-10T00:00Revista brasileira de hematologia e hemoterapia (Online) - Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular (ABHHTC)false
dc.title.none.fl_str_mv Essential thrombocythemia: a rare disease in childhood
title Essential thrombocythemia: a rare disease in childhood
spellingShingle Essential thrombocythemia: a rare disease in childhood
Beatrice,Julia Maimone
Thrombocythemia, essential/drug therapy
Thrombocytosis
Thrombosis
Myelodysplastic-myeloproliferative diseases
Platelet aggregation inhibitors/therapeutic use
Hydroxyurea/therapeutic use
Humans
Case reports
Female
Child
title_short Essential thrombocythemia: a rare disease in childhood
title_full Essential thrombocythemia: a rare disease in childhood
title_fullStr Essential thrombocythemia: a rare disease in childhood
title_full_unstemmed Essential thrombocythemia: a rare disease in childhood
title_sort Essential thrombocythemia: a rare disease in childhood
author Beatrice,Julia Maimone
author_facet Beatrice,Julia Maimone
Garanito,Marlene Pereira
author_role author
author2 Garanito,Marlene Pereira
author2_role author
dc.contributor.author.fl_str_mv Beatrice,Julia Maimone
Garanito,Marlene Pereira
dc.subject.por.fl_str_mv Thrombocythemia, essential/drug therapy
Thrombocytosis
Thrombosis
Myelodysplastic-myeloproliferative diseases
Platelet aggregation inhibitors/therapeutic use
Hydroxyurea/therapeutic use
Humans
Case reports
Female
Child
topic Thrombocythemia, essential/drug therapy
Thrombocytosis
Thrombosis
Myelodysplastic-myeloproliferative diseases
Platelet aggregation inhibitors/therapeutic use
Hydroxyurea/therapeutic use
Humans
Case reports
Female
Child
description Essential thrombocythemia is an acquired myeloproliferative disorder characterized by the proliferation of megakaryocytes in bone marrow, leading to a persistent increase in the number of circulating platelets and thus increasing the risk for thrombotic and hemorrhagic events. The disease features leukocytosis, splenomegaly, vascular occlusive events, hemorrhages and vasomotor disorders. The intricate mechanisms underlying the molecular pathogenesis of this disorder are not completely understood and are still a matter of discussion. Essential thrombocythemia is an extremely rare disorder during childhood. We report on a case of essential thrombocythemia in a child and discuss the diagnostic approach and treatment strategy.
publishDate 2013
dc.date.none.fl_str_mv 2013-01-01
dc.type.driver.fl_str_mv info:eu-repo/semantics/report
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
format report
status_str publishedVersion
dc.identifier.uri.fl_str_mv http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842013000400287
url http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842013000400287
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv 10.5581/1516-8484.20130059
dc.rights.driver.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv text/html
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 Revista Brasileira de Hematologia e Hemoterapia v.35 n.4 2013
reponame:Revista brasileira de hematologia e hemoterapia (Online)
instname:Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular (ABHHTC)
instacron:ABHHTC
instname_str Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular (ABHHTC)
instacron_str ABHHTC
institution ABHHTC
reponame_str Revista brasileira de hematologia e hemoterapia (Online)
collection Revista brasileira de hematologia e hemoterapia (Online)
repository.name.fl_str_mv Revista brasileira de hematologia e hemoterapia (Online) - Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular (ABHHTC)
repository.mail.fl_str_mv sbhh@terra.com.br||secretaria@rbhh.org
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