Mutations in genes encoding isocitrate dehydrogenase (IDH1 and IDH2) in acute myeloid leukemia (AML) result in loss‐of‐function for the oxidative decarboxylation of isocitrate to α‐ketoglutarate (α‐KG) and confer a novel catalytic activity that reduces α‐KG to the D‐enantiomer of 2‐hydroxyglutarate (D‐2HG) [1, 2, 3, 4, 5, 6, 7]. The gene discussed is IDH2; the disease is acute myeloid leukemia.