The most common IDH1 mutation is in the arginine residue at position 132 (IDH1R132), occurring in 6–9% of adult AML, while IDH2 mutations occur in 9–19%, predominantly IDH2R140 in 8–12% [18, 27, 52–54]. IDH1 and IDH2 mutations are mutually exclusive in AML. Here, IDH2 is linked to acute myeloid leukemia.