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Profile Name IDH2 mutant
Gene Variant Detail

IDH2 mutant (unknown)

Relevant Treatment Approaches

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Molecular Profile Indication/Tumor Type Response Type Relevant Treatment Approaches Therapy Name Approval Status Evidence Type Efficacy Evidence References
IDH2 mutant acute myeloid leukemia predicted - sensitive Daunorubicin + Olaparib Preclinical - Patient cell culture Actionable In a preclinical study, the combination therapy of Lynparza (olaparib) and Cerubidine (daunoruibicin) resulted in an additive effect in cells from a patient with acute myeloid leukemia harboring an IDH2 mutation, demonstrating decreased colony formation in culture (PMID: 29339439). 29339439
IDH2 mutant essential thrombocythemia not applicable N/A Guideline Prognostic The presence of at least one mutation in either SH2B3, IDH2, U2AF1, SRSF2, SF3B1, EZH2, TP53, or RUNX1 is associated with inferior overall survival in patients with essential thrombocythemia (NCCN.org). detail...
IDH2 mutant glioblastoma not applicable N/A Guideline Diagnostic IDH2 mutations aid in the diagnosis of secondary grade IV glioblastomas (NCCN.org). detail...
IDH2 mutant malignant astrocytoma not applicable N/A Guideline Diagnostic IDH2 mutations aid in the diagnosis of grade II and grade III astrocytomas (NCCN.org). detail...
IDH2 mutant high grade glioma not applicable N/A Guideline Prognostic IDH2 mutations are associated with a favorable prognosis in patients with glioma, and are associated with a survival benefit for patients treated with radiation or alkylator therapy (NCCN.org). detail...
IDH2 mutant high grade glioma not applicable N/A Guideline Diagnostic IDH2 mutations aid in the diagnosis of gliomas (NCCN.org). detail...
IDH2 mutant angioimmunoblastic T-cell lymphoma not applicable N/A Guideline Diagnostic IDH2 mutations aid in the diagnosis of angioimmunoblastic T-cell lymphoma (NCCN.org). detail...
IDH2 mutant acute myeloid leukemia sensitive Cytarabine + Venetoclax Phase Ib/II Actionable In a Phase I/II trial, Venclexta (venetoclax) in combination with low-dose cytarabine resulted in complete remission or complete remission with incomplete count recovery in 72% (13/18) of patients with acute myeloid leukemia harboring IDH1 or IDH2 mutations who were ineligible for intensive chemotherapy (ASH Annual Meeting, Dec 2018, Abstract 284; NCT02287233). detail...
IDH2 mutant acute myeloid leukemia sensitive Cytarabine + Venetoclax Guideline Actionable Venclexta (venetoclax) in combination with Cytosar-U (cytarabine) is included in guidelines for adult patients with acute myeloid leukemia harboring an IDH2 mutation (NCCN.org). detail...
IDH2 mutant acute myeloid leukemia sensitive Enasidenib FDA approved - Has Companion Diagnostic Actionable In a Phase I/II trial that supported FDA approval, Idhifa (enasidenib) treatment resulted in an overall response rate of 40.3% (71/176) with a median response duration of 5.8 months, complete remission in 19.3% (34/176), and stable disease in 48.3% (85/176) of acute myeloid leukemia patients harboring IDH2 mutations (R140Q/L/G/W, R172K/M/G/S/W) (PMID: 28588020; NCT01915498). 28588020 detail... detail...
IDH2 mutant acute myeloid leukemia sensitive Enasidenib Guideline Actionable Idhifa (enasidenib) is included in guidelines for patients with relapsed or refractory acute myeloid leukemia harboring an IDH2 mutation (NCCN.org). detail...
IDH2 mutant high grade glioma predicted - sensitive AG-881 Case Reports/Case Series Actionable In a Phase I trial, Vorasidenib (AG-881) treatment resulted in an objective response in 13.6% (3/21, 1 partial response, 2 minor response) and stable disease in 77.3% (17/21) of patients with recurrent or progressive non-enhancing glioma harboring mutations in IDH1 (n=20) or IDH2 (n=1), with 60.5% of the patients remained progression-free and alive at 24 months (J Clin Oncol 38: 2020 (suppl; abstr 2504); NCT02481154). detail...
IDH2 mutant acute myeloid leukemia predicted - sensitive Olaparib Preclinical - Patient cell culture Actionable In a preclinical study, cells from a patient with acute myeloid leukemia harboring an IDH2 mutation were sensitive to treatment with Lynparza (olaparib) in culture, demonstrating decreased colony formation (PMID: 29339439). 29339439
IDH2 mutant acute myeloid leukemia predicted - sensitive Venetoclax Phase II Actionable In a Phase II trial, 33% (4/12) of acute myeloid leukemia patients harboring either IDH1 or IDH2 mutations responded to treatment with Venclexta (venetoclax), demonstrating a complete response or complete response with incomplete blood count recovery (PMID: 27520294). 27520294
IDH2 mutant acute myeloid leukemia sensitive Decitabine + Venetoclax Guideline Actionable Venclexta (venetoclax) in combination with Dacogen (decitabine) is included in guidelines for adult patients with acute myeloid leukemia harboring an IDH2 mutation (NCCN.org). detail...
IDH2 mutant acute myeloid leukemia sensitive Azacitidine + Venetoclax Guideline Actionable Venclexta (venetoclax) in combination with Vidaza (azacitidine) is included in guidelines for adult patients with acute myeloid leukemia harboring an IDH2 mutation (NCCN.org). detail...
IDH2 mutant acute myeloid leukemia sensitive Decitabine Guideline Actionable Dacogen (decitabine) is included in guidelines for adult patients with acute myeloid leukemia harboring an IDH2 mutation (NCCN.org). detail...
IDH2 mutant oligodendroglioma not applicable N/A Guideline Diagnostic IDH2 mutations aid in the diagnosis of oligodendrogliomas (NCCN.org). detail...
IDH2 mutant acute myeloid leukemia predicted - sensitive Daunorubicin + Talazoparib Preclinical - Patient cell culture Actionable In a preclinical study, the combination therapy of Talzenna (talazoparib) and Cerubidine (daunoruibicin) resulted in an additive effect in cells from a patient with acute myeloid leukemia harboring an IDH2 mutation, demonstrating decreased colony formation in culture (PMID: 29339439). 29339439
IDH2 mutant acute myeloid leukemia sensitive Azacitidine Guideline Actionable Vidaza (azacitidine) is included in guidelines for adult patients with acute myeloid leukemia harboring an IDH2 mutation (NCCN.org). detail...
IDH2 mutant anaplastic astrocytoma not applicable N/A Guideline Diagnostic IDH2 mutations aid in the diagnosis of grade III astrocytomas (NCCN.org). detail...
IDH2 mutant hematologic cancer sensitive Enasidenib Phase I Actionable In a Phase I study, Enasidenib (AG-221) demonstrated safety and efficacy in patients with hematological cancer harboring IDH2 mutations and included 8 CR, 1 CRp, 3 CRi, and 8 PR (ASH Meeting, Dec 2014, abstract #115). detail...
IDH2 mutant myelofibrosis not applicable N/A Guideline Diagnostic IDH2 mutations aid in the diagnosis of primary myelofibrosis in the absence of JAK2, CALR, or MPL mutations (NCCN.org). detail...
IDH2 mutant myelofibrosis not applicable N/A Guideline Prognostic IDH2 mutations are associated with inferior leukemia-free survival in patients with myelofibrosis (NCCN.org). detail...
IDH2 mutant polycythemia vera not applicable N/A Guideline Prognostic IDH2 mutations are associated with inferior overall survival and leukemia-free survival in patients with polycythemia vera (NCCN.org). detail...
IDH2 mutant acute myeloid leukemia predicted - sensitive Talazoparib Preclinical - Patient cell culture Actionable In a preclinical study, cells from a patient with acute myeloid leukemia harboring an IDH2 mutation were sensitive to treatment with Talzenna (talazoparib) in culture, demonstrating decreased colony formation (PMID: 29339439). 29339439
Clinical Trial Phase Therapies Title Recruitment Status Covered Countries Other Countries
NCT02968940 Phase II Avelumab Avelumab With Hypofractionated Radiation Therapy in Adults With Isocitrate Dehydrogenase (IDH) Mutant Glioblastoma Completed USA 0
NCT04015700 Phase I GNOS-PV01 + INO-9012 Neoantigen-based Personalized DNA Vaccine in Patients With Newly Diagnosed, Unmethylated Glioblastoma Recruiting USA 0
NCT04955938 Phase I Enasidenib + Fedratinib Fedratinib + Ivosidenib A Study of Fedratinib With IDH Inhibition in Advanced-Phase, IDH-Mutated Ph-Negative Myeloproliferative Neoplasms Not yet recruiting USA 0
NCT02492737 Phase I AG-881 Study of Orally Administered AG-881 in Patients With Advanced Hematologic Malignancies With an IDH1 and/or IDH2 Mutation Completed USA 1
NCT03158389 Phase Ib/II Vismodegib Alectinib Idasanutlin Temsirolimus Atezolizumab Palbociclib APG101 NCT Neuro Master Match - N²M² (NOA-20) (N²M²) Recruiting 1
NCT04606316 Phase I Nivolumab Ipilimumab + Nivolumab Surgical Nivolumab And Ipilimumab For Recurrent GBM Recruiting USA 0
NCT03991832 Phase II Durvalumab + Olaparib Study of Olaparib and Durvalumab in IDH-Mutated Solid Tumors Recruiting CAN 0
NCT02273739 Phase Ib/II Enasidenib Study of Orally Administered AG-221 in Subjects With Advanced Solid Tumors, Including Glioma, and With Angioimmunoblastic T-cell Lymphoma, With an IDH2 Mutation Completed USA 1
NCT04729959 Phase II Atezolizumab + Tocilizumab Testing the Addition of the Immune Therapy Drugs, Tocilizumab and Atezolizumab, to Radiation Therapy for Recurrent Glioblastoma Recruiting USA 0
NCT03047993 Phase Ib/II Azacitidine + Telaglenastat Glutaminase Inhibitor CB-839 and Azacitidine in Treating Patients With Advanced Myelodysplastic Syndrome Recruiting USA 0
NCT04774393 Phase Ib/II Decitabine and Cedazuridine + Ivosidenib + Venetoclax Decitabine and Cedazuridine + Enasidenib + Venetoclax Decitabine/Cedazuridine and Venetoclax in Combination With Ivosidenib or Enasidenib for the Treatment of Relapsed or Refractory Acute Myeloid Leukemia Recruiting USA 0
NCT04203316 Phase II Enasidenib Enasidenib for the Treatment of Relapsed or Refractory Acute Myeloid Leukemia Patients With an IDH2 Mutation Recruiting USA | CAN 0
NCT03744390 Phase II Enasidenib IDH2 (AG 221) Inhibitor in Patients With IDH2 Mutated Myelodysplastic Syndrome Recruiting 1
NCT03749187 Phase I Pamiparib + Temozolomide BGB-290 and Temozolomide in Treating Isocitrate Dehydrogenase (IDH)1/2-Mutant Grade I-IV Gliomas (PNOC017) Recruiting USA | CAN 0
NCT02577406 Phase III Azacitidine Cytarabine Enasidenib An Efficacy and Safety Study of AG-221 (CC-90007) Versus Conventional Care Regimens in Older Subjects With Late Stage Acute Myeloid Leukemia Harboring an Isocitrate Dehydrogenase 2 Mutation (IDHENTIFY) Active, not recruiting USA | CAN 16
NCT04945148 Phase II Metformin + Temozolomide Oxidative Phosphorylation Targeting In Malignant Glioma Using Metformin Plus Radiotherapy Temozolomide (OPTIMUM) Not yet recruiting 2
NCT03825796 Phase II CPX-351 + Enasidenib Cytarabine + Enasidenib Enasidenib CPX-351 Plus Enasidenib for Relapsed AML Suspended USA 0
NCT03718767 Phase II Nivolumab Nivolumab in Patients With IDH-Mutant Gliomas With and Without Hypermutator Phenotype Recruiting USA 0
NCT05052957 Phase II Carmustine + Filgrastim + O6-benzylguanine + Temozolomide hSTAR GBM (Hematopoetic Stem Cell (HPC) Rescue for GBM) (hSTAR GBM) Not yet recruiting USA 0
NCT02576444 Phase II Adavosertib + Olaparib Olaparib Capivasertib + Olaparib Ceralasertib + Olaparib OLAParib COmbinations (OLAPCO) Active, not recruiting USA 0
NCT02428855 Phase II Dasatinib Phase II Trial of Dasatinib in Patients With Isocitrate Dehydrogenase (IDH)-Mutant Advanced Intrahepatic Cholangiocarcinoma Completed USA 0
NCT03881735 Phase II Enasidenib Enasidenib in Treating Patients With Relapsed or Refractory Acute Myeloid Leukemia With an IDH2 Gene Mutation Withdrawn USA 0
NCT02632708 Phase I Enasidenib + Etoposide + Mitoxantrone Cytarabine + Enasidenib Cytarabine + Idarubicin + Ivosidenib Cytarabine + Daunorubicin + Enasidenib Cytarabine + Enasidenib + Idarubicin Etoposide + Ivosidenib + Mitoxantrone Cytarabine + Ivosidenib Cytarabine + Daunorubicin + Ivosidenib Safety Study of AG-120 or AG-221 in Combination With Induction and Consolidation Therapy in Participants With Newly Diagnosed Acute Myeloid Leukemia (AML) With an IDH1 and/or IDH2 Mutation Active, not recruiting USA 2
NCT03207347 Phase II Niraparib A Trial of Niraparib in BAP1 and Other DNA Damage Response (DDR) Deficient Neoplasms (UF-STO-ETI-001) Suspended USA 0
NCT02858895 Phase II MDNA55 Convection-Enhanced Delivery (CED) of MDNA55 in Adults With Recurrent or Progressive Glioblastoma Completed USA 0
NCT03212274 Phase II Olaparib Olaparib in Treating Patients With Advanced Glioma, Cholangiocarcinoma, or Solid Tumors With IDH1 or IDH2 Mutations Recruiting USA 0
NCT03557359 Phase II Nivolumab Nivolumab for Recurrent or Progressive IDH Mutant Gliomas Unknown status USA 0
NCT02981940 Phase II Abemaciclib A Study of Abemaciclib in Recurrent Glioblastoma Recruiting USA 0
NCT03701295 Phase Ib/II Azacitidine + Pinometostat Pinometostat and Azacitidine in Treating Patients With Relapsed, Refractory, or Newly Diagnosed Acute Myeloid Leukemia With 11q23 Rearrangement Completed USA 0
NCT04396860 Phase II Temozolomide Ipilimumab + Nivolumab Testing the Use of the Immunotherapy Drugs Ipilimumab and Nivolumab Plus Radiation Therapy Compared to the Usual Treatment (Temozolomide and Radiation Therapy) for Newly Diagnosed MGMT Unmethylated Glioblastoma Recruiting USA 0
NCT02481154 Phase I AG-881 Study of Orally Administered AG-881 in Patients With Advanced Solid Tumors, Including Gliomas, With an IDH1 and/or IDH2 Mutation Active, not recruiting USA 0
NCT04762602 Phase I HMPL-306 A Study of HMPL-306 in Advanced Solid Tumors With IDH Mutations Recruiting USA 0
NCT04555577 Phase I MSC2490484A Temozolomide Nedisertib and Radiation Therapy, Followed by Temozolomide for the Treatment of Patients With Newly Diagnosed MGMT Unmethylated Glioblastoma or Gliosarcoma Recruiting USA 0
NCT01915498 Phase I Enasidenib Phase 1/2 Study of AG-221 in Subjects With Advanced Hematologic Malignancies With an IDH2 Mutation Active, not recruiting USA 1
NCT02071862 Phase I Everolimus + Telaglenastat Erlotinib + Telaglenastat Docetaxel + Telaglenastat Telaglenastat Paclitaxel + Telaglenastat Cabozantinib + Telaglenastat Study of the Glutaminase Inhibitor CB-839 in Solid Tumors Completed USA 0
NCT03728335 Phase I Enasidenib Enasidenib as Maintenance Therapy in Treating Patients With Acute Myeloid Leukemia With IDH2 Mutation After Donor Stem Cell Transplant Recruiting USA 0
NCT03430791 Phase II Ipilimumab + Nivolumab Nivolumab Trial of Combination TTF(Optune), Nivolumab Plus/Minus Ipilimumab for Recurrent Glioblastoma Terminated USA 0
NCT02525692 Phase II ONC201 Oral ONC201 in Recurrent GBM, H3 K27M Glioma, and Midline Glioma Recruiting USA 0
NCT03683433 Phase II Azacitidine + Enasidenib Enasidenib and Azacitidine in Treating Patients With Recurrent or Refractory Acute Myeloid Leukemia and IDH2 Gene Mutation Unknown status USA 0
NCT03878095 Phase II Ceralasertib + Olaparib Testing Olaparib and AZD6738 in IDH1 and IDH2 Mutant Tumors Recruiting USA 0
NCT03139916 Phase II Bavituximab + Temozolomide Bavituximab With Radiation and Temozolomide for Patients With Newly Diagnosed Glioblastoma Active, not recruiting USA 0
NCT04281498 Phase II Enasidenib + Ruxolitinib Combined Ruxolitinib and Enasidenib in Patients With Accelerated/Blast-phase Myeloproliferative Neoplasm or Chronic-phase Myelofibrosis With an IDH2 Mutation Recruiting USA | CAN 0
NCT03922555 Phase I Decitabine and Cedazuridine ASTX727 in Recurrent/Progressive Non-enhancing IDH Mutant Gliomas Recruiting USA 0
NCT04118036 Phase II Abemaciclib + Pembrolizumab Abemaciclib + Pembrolizumab In Glioblastoma Suspended USA 0
NCT04629209 Phase II ONC201 A Phase II, Open Label Study of ONC201 in Adults With EGFR-low Glioblastoma Withdrawn USA 0
NCT02677922 Phase Ib/II Azacitidine + Ivosidenib Azacitidine Azacitidine + Enasidenib A Safety and Efficacy Study of Oral AG-120 Plus Subcutaneous Azacitidine and Oral AG-221 Plus Subcutaneous Azacitidine in Subjects With Newly Diagnosed Acute Myeloid Leukemia (AML) Active, not recruiting USA | CAN 10
NCT04764474 Phase I HMPL-306 A Study of HMPL-306 in Advanced Hematological Malignancies With mIDH Recruiting USA 0