title: Recurrent TET2 mutations in peripheral T-cell lymphomas correlate with TFH-like features and adverse clinical parameters.
abstract: Inactivating mutations of the Ten-Eleven Translocation 2 (TET2) gene were first identified in myeloid malignancies and more recently in peripheral T-cell lymphomas (PTCLs). In the present study, we investigated the presence of TET2 coding sequence mutations and their clinical relevance in a large cohort of 190 PTCL patients. TET2 mutations were identified in 40 of 86 (47%) cases of angioimmunoblastic T-cell lymphoma (AITL) and in 22 of 58 (38%) cases of peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS), but were absent in all other PTCL entities, with the exception of 2 of 10 cases of enteropathy-associated T-cell lymphoma. Among PTCL-NOS, a heterogeneous group of lymphoma-comprising cases likely to derive from Th follicular (T(FH)) cells similarly to AITL, TET2 mutations were more frequent when PTCL-NOS expressed T(FH) markers and/or had features reminiscent of AITL (58% vs 24%, P = .01). In the AITL and PTCL-NOS subgroups, TET2 mutations were associated with advanced-stage disease, thrombocytopenia, high International Prognostic Index scores, and a shorter progression-free survival.
authors: INSERM U955, Université Paris Est, Créteil, France.
authors: François
authors: F
authors: Lemonnier
authors: Lucile
authors: L
authors: Couronné
authors: Marie
authors: M
authors: Parrens
authors: Jean-Philippe
authors: JP
authors: Jaïs
authors: Marion
authors: M
authors: Travert
authors: Laurence
authors: L
authors: Lamant
authors: Olivier
authors: O
authors: Tournillac
authors: Therese
authors: T
authors: Rousset
authors: Bettina
authors: B
authors: Fabiani
authors: Rob A
authors: RA
authors: Cairns
authors: Tak
authors: T
authors: Mak
authors: Christian
authors: C
authors: Bastard
authors: Olivier A
authors: OA
authors: Bernard
authors: Laurence
authors: L
authors: de Leval
authors: Philippe
authors: P
authors: Gaulard
mesh_terms: D000368:Aged; D004268:DNA-Binding Proteins / Q000235:genetics*; D049308:Dioxygenases; D005260:Female; D006801:Humans; D016411:Lymphoma, T-Cell, Peripheral / Q000235:genetics* / Q000276:immunology*; D008297:Male; D009154:Mutation / Q000235:genetics*; D011518:Proto-Oncogene Proteins / Q000235:genetics*; D012008:Recurrence; D006377:T-Lymphocytes, Helper-Inducer / Q000276:immunology* / Q000473:pathology*
chemical_list: D004268:DNA-Binding Proteins; D011518:Proto-Oncogene Proteins; D049308:Dioxygenases; C541081:TET2 protein, human