Biomarker · Gene alteration

IDH2 mutation

IDH2 codon 140 or 172 mutations detected by PCR or sequencing of blood or marrow; used as a predictive marker for IDH2 inhibitors in acute myeloid leukemia and as a diagnostic marker in glioma.

CI-BIO-00000048IDH2 graph →NCIt C118390

Also calledIDH2-mutated · R140Q · R172K

Derived

Associated cancers (8)

Cancers mapped in ACCEPTED CIViC predictive, prognostic or diagnostic items whose variant (molecular markers) or gene (gene-level markers) is in this biomarker's scope. Counts by type and native level; each row links to the cancer's evidence tab.

Computedrule biomarker-links-v1

Cancer	Items	Predictive	Prognostic	Diagnostic	Levels A / B / C / D / E
Acute Myeloid Leukemia	10	3	7	—	1 / 7 / 1 / 1 / 0
Myelodysplastic Syndrome	3	—	3	—	0 / 3 / 0 / 0 / 0
Peripheral T-Cell Lymphoma, Not Otherwise Specified	2	—	1	1	0 / 2 / 0 / 0 / 0
Anaplastic Astrocytoma	1	—	1	—	0 / 1 / 0 / 0 / 0

Derived

Drugs with predictive evidence (3)

Therapies named in in-scope PREDICTIVE CIViC items, plus targets of PREDICTS_RESPONSE_TO knowledge edges from in-scope variants. Sensitivity and resistance are counted separately and never merged into a verdict.

Computedrule biomarker-links-v1

Drug	CIViC items	Sensitivity	Resistance	Best level	Edges (S / R)	Cancer context
VorasidenibSmall Molecule	2	2	—	A	4 / 0	Glioma, Low Grade Glioma
EnasidenibSmall Molecule	2	2	—	A	2 / 0	Acute Myeloid Leukemia
Venetoclax

Curated evidence

Predictive evidence items (5)

ACCEPTED CIViC predictive items in scope, grouped by molecular profile and therapy with native level, direction and cancer context. 50 per page.

civicCuratedShowing 1–5 of 5 evidence items · levels, directions and significance as curated at the source; each row links to its CIViC record.

Therapy	Cancer	Type	Level	Direction · significance	Rating (1–5)	Status	Evidence	Source
IDH2 Mutation3
Enasidenib

Regulatory

Approvals mentioning the linked drugs (26)

Approval records of the derived drug set, plus records whose indication text names this biomarker. Each record names its authority, jurisdiction, indication text and status — a drug approved in one jurisdiction for one indication is not 'approved' in general, and an approval listed here is not necessarily restricted to this biomarker.

Computedrule biomarker-links-v1

health-canada-dpd

Clinical trials

Active trials (174)

Trials with an active ClinicalTrials.gov status, an intervention mapped to a derived drug and a condition mapped to an associated cancer. 110 recruiting · 26 active phase 3 · 279 in any status. Most recently updated first, 50 per page.

Computedrule biomarker-links-v1

clinicaltrialsPublishedShowing 1–50 of 174 active studies · status and phase as posted by the registrant; each row links to the study record.

NCT	Title	Status	Phase	Enrollment (n)	Sponsor	Countries	Last update	Source
NCT05554393	Comparing Cytarabine + Daunorubicin Therapy Versus Cytarabine + Daunorubicin + Venetoclax Versus Venetoclax + Azacitidine in Younger Patients With Intermediate Risk AML (A MyeloMATCH Treatment Trial)	recruiting

Literature

Linked publications (15)

Publications linked to the scope gene(s) through PubMed entity edges; 25 per page, newest first.

pubmedPublishedShowing 1–15 of 15 publications · bibliographic data as recorded by PubMed; abstracts are not reproduced.

Vorasidenib in IDH1- or IDH2-Mutant Low-Grade Glioma.
· N Engl J Med · 2023 · PMID 37272516 · validated civic_curation pubmed
Enasidenib in mutant IDH2 relapsed or refractory acute myeloid leukemia.
· Blood · 2017 · PMID 28588020 · validated civic_curation pubmed
IDH1 and IDH2 mutations in gliomas.
· N Engl J Med · 2009 · PMID 19228619 · validated civic_curation pubmed
Genomic Classification and Prognosis in Acute Myeloid Leukemia.
· N Engl J Med · 2016 · PMID 27276561 · validated civic_curation pubmed

IDH1 Mutation + IDH2 MutationIDH1 IDH21
Vorasidenib	Glioma	Predictive	A	Supports Sensitivity Response	4	accepted	EID11708 In this study, 331 patients with IDH-mutant grade 2 gliomas were either given vorasidenib (n = 168) or a placebo (n = 163). A median PFS of 27.7 months was reported in the vorasidenib group, compared … (full text at CIViC) PMID 37272516 · Mellinghoff et al., 2023 · Open in CIViC	civic

IDH1 Mutation + IDH2 MutationIDH1 IDH21

Vorasidenib

Glioma

Predictive

Supports Sensitivity Response

accepted

EID11708

In this study, 331 patients with IDH-mutant grade 2 gliomas were either given vorasidenib (n = 168) or a placebo (n = 163). A median PFS of 27.7 months was reported in the vorasidenib group, compared … (full text at CIViC)

PMID 37272516 · Mellinghoff et al., 2023 · Open in CIViC

civic

IDH2 R172G + IDH2 R172K + IDH2 R172W1
Vorasidenib	Low Grade Glioma	Predictive	A	Supports Sensitivity Response	5	accepted	EID11509 This phase 3 clinical trial was conducted to assess the progression-free survival in patients with IDH-mutant gliomas treated with Vorasidenib, an oral brain-penetrant inhibitor, which showed prelimin… (full text at CIViC) PMID 37272516 · Mellinghoff et al., 2023 · Open in CIViC	civic

IDH2 R172G + IDH2 R172K + IDH2 R172W1

Vorasidenib

Low Grade Glioma

Predictive

Supports Sensitivity Response

accepted

EID11509

This phase 3 clinical trial was conducted to assess the progression-free survival in patients with IDH-mutant gliomas treated with Vorasidenib, an oral brain-penetrant inhibitor, which showed prelimin… (full text at CIViC)

PMID 37272516 · Mellinghoff et al., 2023 · Open in CIViC

civic

Drug	Jurisdiction · authority	Cancer	Indication	Status	Approval date	Source
Enasidenib	CA Health Canada	—	Marketed in Canada as IDHIFA (DIN 02485427) under ATC L01XM01 ENASIDENIB. Indications are not published in the Drug Product Database — see the Health Canada Product Monograph.	withdrawnsource: Cancelled Post Marketsince Jun 30, 2023	Feb 27, 2019	health-canada-dpd
Enasidenib	CA Health Canada	—	Marketed in Canada as IDHIFA (DIN 02485435) under ATC L01XM01 ENASIDENIB. Indications are not published in the Drug Product Database — see the Health Canada Product Monograph.	withdrawnsource: Cancelled Post Marketsince Jun 30, 2023	Feb 27, 2019	health-canada-dpd
Enasidenib	US FDA	—	Efficacy supplement 2020-11-24 (see label)	approved	Nov 24, 2020	openfda
Enasidenib	US FDA	Acute Myeloid Leukemia	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	approved	Aug 1, 2017	openfda
Venetoclax	CA Health Canada	—	Marketed in Canada as VENCLEXTA (DIN 02458055) under ATC L01XX52 VENETOCLAX. Indications are not published in the Drug Product Database — see the Health Canada Product Monograph.	approvedsource: Marketed	Oct 31, 2016	health-canada-dpd
Venetoclax	CA Health Canada	—	Marketed in Canada as VENCLEXTA (DIN 02458047) under ATC L01XX52 VENETOCLAX. Indications are not published in the Drug Product Database — see the Health Canada Product Monograph.	approvedsource: Marketed	Oct 31, 2016	health-canada-dpd
Venetoclax	CA Health Canada	—	Marketed in Canada as VENCLEXTA (DIN 02458039) under ATC L01XX52 VENETOCLAX. Indications are not published in the Drug Product Database — see the Health Canada Product Monograph.	approvedsource: Marketed	Oct 31, 2016	health-canada-dpd
Venetoclax	CA Health Canada	—	Marketed in Canada as VENCLEXTA (DIN 02458063) under ATC L01XX52 VENETOCLAX. Indications are not published in the Drug Product Database — see the Health Canada Product Monograph.	approvedsource: Marketed	Oct 31, 2016	health-canada-dpd
Venetoclax	EU EMA	—	Venclyxto is indicated for the treatment of adult patients with previously untreated chronic lymphocytic leukaemia (CLL): in combination with acalabrutinib with or without obinutuzumab  in combination with obinutuzumab in combination with ibrutinib   Venclyxto in combination with rituximab is indicated for the treatment of adult patients with CLL who have received at least one prior therapy.   Venclyxto monotherapy is indicated for the treatment of CLL: in the presence of 17p deletion or TP53 mutation in adult patients who are unsuitable for or have failed a B cell receptor pathway inhibitor, or in the absence of 17p deletion or TP53 mutation in adult patients who have failed both chemoimmunotherapy and a B cell receptor pathway inhibitor.   Venclyxto in combination with a hypomethylating agent is indicated for the treatment of adult patients with newly diagnosed acute myeloid leukaemia (AML) who are ineligible for intensive chemotherapy.	approved	Dec 4, 2016	ema
Venetoclax	US FDA	—	Efficacy supplement 2026-05-20 (see label)	approved	May 20, 2026	openfda
Venetoclax	US FDA	—	Efficacy supplement 2026-02-19 (see label)	approved	Feb 19, 2026	openfda
Venetoclax	US FDA	—	Efficacy supplement 2021-10-04 (see label)	approved	Oct 4, 2021	openfda
Venetoclax	US FDA	—	Efficacy supplement 2020-10-16 (see label)	approved	Oct 16, 2020	openfda
Venetoclax	US FDA	—	Efficacy supplement 2020-10-16 (see label)	approved	Oct 16, 2020	openfda
Venetoclax	US FDA	—	Efficacy supplement 2019-05-15 (see label)	approved	May 15, 2019	openfda
Venetoclax	US FDA	—	Efficacy supplement 2018-11-21 (see label)	approved	Nov 21, 2018	openfda
Venetoclax	US FDA	—	Efficacy supplement 2018-09-07 (see label)	approved	Sep 7, 2018	openfda
Venetoclax	US FDA	—	Efficacy supplement 2018-06-08 (see label)	approved	Jun 8, 2018	openfda
Venetoclax	US FDA	—	Efficacy supplement 2018-06-08 (see label)	approved	Jun 8, 2018	openfda
Venetoclax	US FDA	—	Efficacy supplement 2017-12-20 (see label)	approved	Dec 20, 2017	openfda
Venetoclax	US FDA	—	For the treatment of adult patients with chronic lymphocytic leukemia (CLL) or small lymphocytic lymphoma (SLL).	approved	Apr 11, 2016	openfda
Venetoclax	US FDA	Acute Myeloid Leukemia	In combination with azacitidine, or decitabine, or low-dose cytarabine for the treatment of newly diagnosed acute myeloid leukemia (AML) in adults 75 years or older, or who have comorbidities that preclude use of intensive induction chemotherapy.	approved	Apr 11, 2016	openfda
Vorasidenib	CA Health Canada	—	Marketed in Canada as VORANIGO (DIN 02551233) under ATC L01XM04 VORASIDENIB. Indications are not published in the Drug Product Database — see the Health Canada Product Monograph.	approvedsource: Marketed	Oct 21, 2024	health-canada-dpd
Vorasidenib	CA Health Canada	—	Marketed in Canada as VORANIGO (DIN 02551225) under ATC L01XM04 VORASIDENIB. Indications are not published in the Drug Product Database — see the Health Canada Product Monograph.	approvedsource: Marketed	Oct 21, 2024	health-canada-dpd
Vorasidenib	EU EMA	Grade 2 Follicular Lymphoma	Voranigo as monotherapy is indicated for the treatment of predominantly non‑enhancing Grade 2 astrocytoma or oligodendroglioma with an IDH1 R132 or IDH2 R172 mutation in adult and adolescent patients aged 12 years and older and weighing at least 40 kg who only had surgical intervention and are not in immediate need of radiotherapy or chemotherapy (see section 5.1).	approved	Sep 17, 2025	ema
Vorasidenib	US FDA	Grade 2 Follicular Lymphoma	VORANIGO is indicated for the treatment of adult and pediatric patients 12 years and older with Grade 2 astrocytoma or oligodendroglioma with a susceptible isocitrate dehydrogenase-1 (IDH1) or isocitrate dehydrogenase-2 (IDH2) mutation, as detected by an FDA-approved test, following surgery including biopsy, sub-total resection, or gross total resection [see Dosage and Administration (2.1) , Clinical Pharmacology (12.1) and Clinical Studies (14) ]. VORANIGO is an isocitrate dehydrogenase-1 (IDH1) and isocitrate dehydrogenase-2 (IDH2) inhibitor indicated for the treatment of adult and pediatric patients 12 years and older with Grade 2 astrocytoma or oligodendroglioma with a susceptible IDH1 or IDH2 mutation, as detected by an FDA-approved test, following surgery including biopsy, sub-total resection, or gross total resection. ( 1 )	approved	Aug 6, 2024	openfda

Drug

Jurisdiction · authority

Cancer

Indication

Status

Approval date

Source

Enasidenib

CA Health Canada

—

Marketed in Canada as IDHIFA (DIN 02485427) under ATC L01XM01 ENASIDENIB. Indications are not published in the Drug Product Database — see the Health Canada Product Monograph.

withdrawnsource: Cancelled Post Marketsince Jun 30, 2023

Feb 27, 2019

health-canada-dpd

Enasidenib

CA Health Canada

—

Marketed in Canada as IDHIFA (DIN 02485435) under ATC L01XM01 ENASIDENIB. Indications are not published in the Drug Product Database — see the Health Canada Product Monograph.

withdrawnsource: Cancelled Post Marketsince Jun 30, 2023

Feb 27, 2019

health-canada-dpd

Enasidenib

US FDA

—

Efficacy supplement 2020-11-24 (see label)

approved

Nov 24, 2020

openfda

Enasidenib

US FDA

Acute Myeloid Leukemia

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

approved

Aug 1, 2017