Among its three isoforms, mutations in the IDH1 and IDH2 genes are found in the majority of low- and high-grade gliomas [94] as well as in 20% of adults with acute myeloid leukemia (AML), and 5% of adults with myelodysplastic syndromes (MDSs) [166]. Here, IDH2 is linked to acute myeloid leukemia.