Understanding triple negative myeloproliferative neoplasms: pathogenesis, clinical features, and management.
Leuk Lymphoma
; 65(2): 158-167, 2024 Feb.
Article
em En
| MEDLINE
| ID: mdl-38033130
ABSTRACT
ABSTRACTMyeloproliferative neoplasms (MPNs) that lack the classical "driver mutations," termed triple negative MPNs, remain a poorly understood entity. Despite considerable progress toward understanding MPN pathobiology, the mechanisms leading to the development of these MPNs remains inadequately elucidated. While triple negative primary myelofibrosis (TN-PMF) portends a poor prognosis, triple negative essential thrombocythemia (TN-ET) is more favorable as compared with JAK2 mutated ET. In this review, we summarize the clinical features and prognosis of TN-PMF and -ET as well as diagnostic challenges including identification of non-canonical driver mutations. We also discuss additional molecular drivers to better understand possible pathogenic mechanisms underlying triple negative MPNs. Finally, we highlight current therapeutic approaches as well as novel targets, particularly in the difficult to treat TN-PMF population.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Temas:
Geral
/
Tipos_de_cancer
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Outros_tipos
Base de dados:
MEDLINE
Assunto principal:
Mielofibrose Primária
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Trombocitemia Essencial
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Transtornos Mieloproliferativos
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Neoplasias
Limite:
Humans
Idioma:
En
Revista:
Leuk Lymphoma
Assunto da revista:
HEMATOLOGIA
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NEOPLASIAS
Ano de publicação:
2024
Tipo de documento:
Article
País de afiliação:
Estados Unidos