title: The curious origins of angioimmunoblastic T-cell lymphoma.
abstract: 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.
  
  
  
  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.
  
  
  
  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.
authors.affiliation: aInstitute for Cancer Genetics, Columbia University bDepartment of Pathology and Cell Biology, Columbia University Medical Center, New York, USA.
authors.forename: José R
authors.initials: JR
authors.lastname: Cortés
authors.forename: Teresa
authors.initials: T
authors.lastname: Palomero
mesh_terms: D000818:Animals; D014408:Biomarkers, Tumor; D044127:Epigenesis, Genetic; D020869:Gene Expression Profiling; D015972:Gene Expression Regulation, Neoplastic; D020022:Genetic Predisposition to Disease; D023281:Genomics / Q000379:methods; D006801:Humans; D007119:Immunoblastic Lymphadenopathy / Q000175:diagnosis / Q000209:etiology* / Q000378:metabolism; D016399:Lymphoma, T-Cell / Q000175:diagnosis / Q000209:etiology* / Q000378:metabolism; D009154:Mutation; D011948:Receptors, Antigen, T-Cell / Q000378:metabolism; D015398:Signal Transduction; D006377:T-Lymphocytes, Helper-Inducer / Q000378:metabolism / Q000473:pathology; D059467:Transcriptome; D020742:rhoA GTP-Binding Protein / Q000235:genetics
chemical_list: D014408:Biomarkers, Tumor; D011948:Receptors, Antigen, T-Cell; D020742:rhoA GTP-Binding Protein