Resumen
Recent advances in exome sequencing and comprehensive genomic studies have shed new light on the pathogenesis and mutational landscape of Angioimmunoblastic T-cell lymphoma (AITL). The essential pathogenic role of recurrent TET2, DNMT3A, IDH2, and RHOA mutations in AITL has been identified. Notably, the detection of TET2 mutations in bystander B cells in AITL samples has allowed new mechanistic advances in AITL combined with B-cell lymphoma. In this review, we highlight the current role of TET2 mutations in the pathogenesis of AITL and how mutational crosstalk in TET2 and other partner genes (RHOA, DNMT3A, and IDH2) cooperate to contribute to the disease. In addition, we elaborate on recent advances in AITL frequently comprising B-cell lymphoma and discuss the potential prospects of targeting TET2 as an anti-AITL agent.