Multiple combinations of enasidenib/ivosidenib with venetoclax (NCT04092179), decitabine (NCT05010772), decitabine and venetoclax (NCT04774393), and classical induction/consolidation therapy (NCT03839771) are currently under investigation along with the role of IDH inhibitors in maintenance therapy following ASCT in AML (NCT03728335, NCT04522895); in IDH2-mutated MDS (NCT03744390); in association with CPX-351, fedratinib (NCT04955938), and ruxolitinib (NCT04281498); and in IDH1/2-mutated high risk myeloproliferative syndromes. The gene discussed is IDH1; the disease is myelodysplastic syndrome.