{"entity":{"id":"idh2-mutation","kind":"biomarker","name":"IDH2 mutation (R140 and R172)","aka":["IDH2 R140Q","IDH2 R172K","IDH2 mutation","IDH2-mutant","mIDH2"],"tldr":"IDH2 mutations at codons 140 and 172 do the same job as IDH1 R132, producing 2-HG. Enasidenib is approved for relapsed AML with them, and vorasidenib for grade 2 gliomas with either IDH gene mutated.","summary":"R140Q is the commonest IDH2 mutation in AML; R172K predominates in glioma and angioimmunoblastic T-cell lymphoma. Enasidenib (Idhifa, 2017) is labelled for relapsed or refractory AML with an IDH2 mutation as detected by an FDA-approved test, the Abbott RealTime IDH2 assay listing R140Q, R140L, R140G, R140W, R172K, R172M, R172G, R172S and R172W; vorasidenib covers IDH2 R172 mutations in grade 2 glioma with the Oncomine Dx Target Test. IDH2-mutant AML has a lower rate of differentiation syndrome recognition problems only because the drug is older; the risk is the same class effect.","asOf":"2026-09-23","links":[{"label":"Idhifa prescribing information (DailyMed)","url":"https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=a5b4cdf0-3fa8-4c6c-80f6-8d8a00e3a5b6"}],"tags":["biomarker","idh"],"related":["idh1-r132"],"cancers":["aml","aml-idh","idh-mutant-astrocytoma","oligodendroglioma"],"sections":[],"technologies":[],"targets":[],"drugs":["enasidenib","vorasidenib"],"companies":[],"institutions":[],"pathways":[],"terms":["ngs"],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"target":"idh","measurement":"sequencing-variant","scoringRule":{"text":"An IDH2 codon 140 or 172 substitution (R140Q, R140L, R140G, R140W, R172K, R172M, R172G, R172S, R172W) by PCR or sequencing.","quote":"R140Q, R140L, R140G, R140W, R172K, R172M, R172G, R172S, and R172W","source":"https://www.fda.gov/medical-devices/in-vitro-diagnostics/list-cleared-or-approved-companion-diagnostic-devices-in-vitro-and-imaging-tools","sourceLabel":"FDA: List of FDA-Authorized Companion Diagnostic Devices"},"thresholds":[{"value":"IDH2 mutation","drugId":"enasidenib","cancerId":"aml-idh","regulator":"FDA","source":"https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=a5b4cdf0-3fa8-4c6c-80f6-8d8a00e3a5b6","quote":"IDHIFA is an isocitrate dehydrogenase-2 inhibitor indicated for the treatment of adult patients with relapsed or refractory acute myeloid leukemia (AML) with an isocitrate dehydrogenase-2 (IDH2) mutation as detected by an FDA-approved test","status":"current"},{"value":"Susceptible IDH1 or IDH2 mutation, grade 2 glioma","drugId":"vorasidenib","cancerId":"oligodendroglioma","regulator":"FDA","source":"https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=31405fee-55b7-4857-987e-2724ee76be84","quote":"Grade 2 astrocytoma or oligodendroglioma with a susceptible IDH1 or IDH2 mutation, as detected by an FDA-approved test","status":"current"}],"tests":["foundationone-heme","tempus-xt","neotype-profiles"],"assays":["idh2-abbott","oncomine-dx"],"companionDiagnostics":[{"device":"Abbott RealTime IDH2","maker":"Abbott Molecular","companyId":"abbott","drugs":["enasidenib"],"indication":"Acute Myeloid Leukemia - Peripheral Blood or Bone Marrow","pma":"P170005 (08/01/2017)","source":"https://www.fda.gov/medical-devices/in-vitro-diagnostics/list-cleared-or-approved-companion-diagnostic-devices-in-vitro-and-imaging-tools"}],"forPatient":"An IDH2 mutation in acute myeloid leukaemia that has come back opens enasidenib tablets; in a grade 2 glioma after surgery it opens vorasidenib. Both drugs can cause differentiation syndrome in leukaemia, a reaction your team will watch for in the first weeks."},"route":"/biomarkers/idh2-mutation/","neighbours":{"biomarker":[{"id":"1p19q-codeletion-readout","kind":"biomarker","name":"1p/19q codeletion","route":"/biomarkers/1p19q-codeletion-readout/"},{"id":"idh1-r132","kind":"biomarker","name":"IDH1 R132 mutation","route":"/biomarkers/idh1-r132/"}],"cancer":[{"id":"aml","kind":"cancer","name":"Acute myeloid leukaemia","route":"/cancers/aml/"},{"id":"idh-mutant-astrocytoma","kind":"cancer","name":"Astrocytoma, IDH-mutant (grades 2 to 4)","route":"/cancers/idh-mutant-astrocytoma/"},{"id":"aml-idh","kind":"cancer","name":"IDH1- and IDH2-mutated acute myeloid leukaemia","route":"/cancers/aml-idh/"},{"id":"oligodendroglioma","kind":"cancer","name":"Oligodendroglioma, IDH-mutant and 1p/19q-codeleted","route":"/cancers/oligodendroglioma/"}],"drug":[{"id":"enasidenib","kind":"drug","name":"Enasidenib","route":"/drugs/enasidenib/"},{"id":"vorasidenib","kind":"drug","name":"Vorasidenib","route":"/drugs/vorasidenib/"}],"term":[{"id":"ngs","kind":"term","name":"Next-generation sequencing (NGS)","route":"/terms/ngs/"}],"target":[{"id":"idh","kind":"target","name":"IDH1 / IDH2","route":"/targets/idh/"}]}}