In a phase Ib/II trial of 27 patients with r/r IDH2-mutated AML, the combination of enasidenib and venetoclax was well tolerated, with common grade III-V adverse events being febrile neutropenia (41%), thrombocytopenia (26%), and hyperbilirubinemia (15%). Here, IDH2 is linked to acute myeloid leukemia.