Philadelphia-negative Chronic Myeloproliferative Neoplasms

dc.creatorBittencourt R.I.
dc.creatorVassallo J.
dc.creatorChauffaille M.L.L.F.
dc.creatorXavier S.G.
dc.creatorPagnano K.B.
dc.creatorNascimento A.C.K.
dc.creatorde Souza C.A.
dc.creatorChiattone C.S.
dc.date2012
dc.date2015-06-25T20:23:13Z
dc.date2015-11-26T15:18:32Z
dc.date2015-06-25T20:23:13Z
dc.date2015-11-26T15:18:32Z
dc.date.accessioned2018-03-28T22:28:10Z
dc.date.available2018-03-28T22:28:10Z
dc.identifier
dc.identifierRevista Brasileira De Hematologia E Hemoterapia. , v. 34, n. 2, p. 140 - 149, 2012.
dc.identifier15168484
dc.identifier10.5581/1516-8484.20120034
dc.identifierhttp://www.scopus.com/inward/record.url?eid=2-s2.0-84861448274&partnerID=40&md5=ec4536efa920227f5e246df8d18d6cb6
dc.identifierhttp://www.repositorio.unicamp.br/handle/REPOSIP/89995
dc.identifierhttp://repositorio.unicamp.br/jspui/handle/REPOSIP/89995
dc.identifier2-s2.0-84861448274
dc.identifier.urihttp://repositorioslatinoamericanos.uchile.cl/handle/2250/1259594
dc.descriptionChronic myeloproliferative diseases without the Philadelphia chromosome marker (Ph-), although first described 60 years ago, only became the subject of interest after the turn of the millennium. In 2001, the World Health Organization (WHO) defined the classification of this group of diseases and in 2008 they were renamed myeloproliferative neoplasms based on morphological, cytogenetic and molecular features. In 2005, the identification of a recurrent molecular abnormality characterized by a gain of function with a mutation in the gene encoding Janus kinase 2 (JAK2) paved the way for greater knowledge of the pathophysiology of myeloproliferative neoplasms. The JAK2 mutation is found in 90-98% of polycythemia vera and in about 50% essential thrombocytosis and primary myelofibrosis. In addition to the JAK2 mutation, other mutations involving TET2 (ten-eleven translocation), LNK (a membrane-bound adaptor protein); IDH1/2 (isocitrate dehydrogenase 1/2 enzyme); ASXL1 (additional sex combs-like 1) genes were found in myeloproliferative neoplasms thus showing the importance of identifying molecular genetic alterations to confirm diagnosis, guide treatment and improve our understanding of the biology of these diseases. Currently, polycythemia vera, essential thrombocytosis, myelofibrosis, chronic neutrophilic leukemia, chronic eosinophilic leukemia and mastocytosis are included in this group of myeloproliferative neoplasms, but are considered different situations with individualized diagnostic methods and treatment. This review updates pathogenic aspects, molecular genetic alterations, the fundamental criteria for diagnosis and the best approach for each of these entities.
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dc.languageen
dc.publisher
dc.relationRevista Brasileira de Hematologia e Hemoterapia
dc.rightsaberto
dc.sourceScopus
dc.titlePhiladelphia-negative Chronic Myeloproliferative Neoplasms
dc.typeArtículos de revistas


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