This allowed a better understanding of different biologic pathways involved in AITL pathogenesis Recurrent genetic abnormalities in ras homolog family member A (RHOA) (50–70%) and in genes of the epigenetic modulators, tet methyl cytosine dioxygenase 2 (TET2) (47–83%), DNA methyltransferase 3 alpha (DNMT3A) (20–30%), isocitrate dehydrogenase 2 (IDH2) (20–45%) have been described [23,24]. This evidence concerns the gene IDH2 and angioimmunoblastic T-cell lymphoma.