# IDH2 mutation (R140 and R172)

Source: https://onco.cc/biomarkers/idh2-mutation/  
OnCo record `idh2-mutation` (Biomarker). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

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.

## Fields

- Kind: Biomarker
- Last checked: 2026-09-23
- Also known as: IDH2 R140Q; IDH2 R172K; IDH2 mutation; IDH2-mutant; mIDH2
- Tags: biomarker; idh

## Sources

- Idhifa prescribing information (DailyMed): https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=a5b4cdf0-3fa8-4c6c-80f6-8d8a00e3a5b6

## Connected records

- biomarkers: [1p/19q codeletion](https://onco.cc/biomarkers/1p19q-codeletion-readout/), [IDH1 R132 mutation](https://onco.cc/biomarkers/idh1-r132/)
- cancers: [Acute myeloid leukaemia](https://onco.cc/cancers/aml/), [Astrocytoma, IDH-mutant (grades 2 to 4)](https://onco.cc/cancers/idh-mutant-astrocytoma/), [IDH1- and IDH2-mutated acute myeloid leukaemia](https://onco.cc/cancers/aml-idh/), [Oligodendroglioma, IDH-mutant and 1p/19q-codeleted](https://onco.cc/cancers/oligodendroglioma/)
- drugs: [Enasidenib](https://onco.cc/drugs/enasidenib/), [Vorasidenib](https://onco.cc/drugs/vorasidenib/)
- terms: [Next-generation sequencing (NGS)](https://onco.cc/terms/ngs/)
- targets: [IDH1 / IDH2](https://onco.cc/targets/idh/)

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