Although the approved IDH2/R140Q inhibitor enasidenib exhibits encouraging therapeutic effects in patients with IDH2/R140Q mutated AML [8], acquired resistance to enasidenib has been reported [9], and approximately 12% of enasidenib-treated patients developed IDH differentiation syndrome, a potentially lethal clinical entity [10]. Here, IDH1 is linked to acute myeloid leukemia.