Abstract: Angioimmunoblastic T-cell lymphoma (AITL) is one of the most common subtypes of peripheral T-cell lymphomas. It is a follicular T-helper-derived neoplasm, and characterized by the presence of some genetic alterations, such as mutations in TET2, DNMT3A, IDH2, and RHOA. Anthracycline-containing regimens represent the most widely adopted first-line option; however, new biologic agents should be designed and incorporated to improve treatment response. The elucidation of the specific roles of these genetic alterations in AITL will facilitate the development of molecularly targeted therapies to treat this disease.