A pivotal study by Stein and coworkers in relapsed or refractory IDH2 mutant AML patients reported an overall response rate of 40.3%, with a median OS of 9.3 months (19.7 months in patients who attained a CR); responses were associated with the cellular differentiation of leukemic blasts and cytotoxicity was not the main driver of the antileukemic activity of Enasidenib [51]. Here, IDH2 is linked to acute myeloid leukemia.