Specific inhibitors for IDH1 (AG-120 or ivosidenib, IDH305, BAY143602, FT-2102), IDH2 (AG-221 or enasidenib) or both IDH1/2 (AG-881) have been proven to be effective and approved or currently in clinical trials, being enasidenib and ivosidenib the first mutant-IDH FDA-approved for AML patients in 2017. Here, IDH2 is linked to acute myeloid leukemia.