Origins of leukaemia in children with Down syndrome.
Nat Rev Cancer
; 5(1): 11-20, 2005 Jan.
Article
em En
| MEDLINE
| ID: mdl-15630411
ABSTRACT
Transient megakaryoblastic leukaemia is found in 10% of newborns with Down syndrome, characterized by constitutional trisomy 21. Although in most cases the leukaemic cells disappear spontaneously after the first months of life, irreversible acute megakaryoblastic leukaemia develops in 20% of these individuals within 4 years. The leukaemic cells typically harbour somatic mutations of the gene encoding GATA1, an essential transcriptional regulator of normal megakaryocytic differentiation. Leukaemia that specifically arises in the context of constitutional trisomy 21 and somatic GATA1 mutations is a unique biological model of the incremental process of leukaemic transformation.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Leucemia Megacarioblástica Aguda
/
Síndrome de Down
Tipo de estudo:
Prognostic_studies
Limite:
Child
/
Child, preschool
/
Humans
/
Infant
/
Newborn
Idioma:
En
Revista:
Nat Rev Cancer
Ano de publicação:
2005
Tipo de documento:
Article