The curious origins of angioimmunoblastic T-cell lymphoma.
Curr Opin Hematol
; 23(4): 434-43, 2016 07.
Article
em En
| MEDLINE
| ID: mdl-27177312
ABSTRACT
PURPOSE OF REVIEW Once an obscure disease, recent studies have transformed our understanding of angioimmunoblastic T-cell lymphoma (AITL). In this review, we summarize new major advances in the genetics and biology of AITL. RECENT FINDINGS:
Genome wide sequencing studies have dissected the repertoire of the genetic alterations driving AITL uncovering a highly recurrent Gly17Val somatic mutation in the small GTPase RHOA and major role for mutations in epigenetic regulators, such as TET2, DNMT3A and IDH2, and signaling factors (e.g., FYN and CD28). These findings support a multistep model of follicular T helper cell transformation in AITL and pinpoint novel candidates for the development of targeted therapies in this disease.SUMMARY:
AITL originates from follicular T helper cells and is characterized by the presence of RHOA G17V mutation together with genetic alterations in TET2, DNMT3A, and IDH2. Research efforts now focus on the elucidation of the specific roles and interplay of these genetic alterations in the pathogenesis of AITL.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Linfoma de Células T
/
Linfadenopatia Imunoblástica
Tipo de estudo:
Diagnostic_studies
/
Prognostic_studies
Limite:
Animals
/
Humans
Idioma:
En
Revista:
Curr Opin Hematol
Assunto da revista:
HEMATOLOGIA
Ano de publicação:
2016
Tipo de documento:
Article
País de afiliação:
Estados Unidos