Missing content? – Request curation!
Request curation for specific Genes, variants, or PubMed publications.
Have questions, comments or suggestions? - Let us know!
Email us at : ckbsupport@jax.org
| Profile Name | IDH2 mutant |
| Gene Variant Detail | |
| Relevant Treatment Approaches |
| 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 | 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 | high grade glioma | not applicable | N/A | Guideline | Diagnostic | IDH2 mutations aid in the diagnosis of gliomas (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 | 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 | 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 | 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 | 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 | glioblastoma | not applicable | N/A | Guideline | Diagnostic | IDH2 mutations aid in the diagnosis of secondary grade IV glioblastomas (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 | 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 | 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 | 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 | 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 | 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 | 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 | 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 | |
| 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 | 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 | 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 | 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 | 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 | oligodendroglioma | not applicable | N/A | Guideline | Diagnostic | IDH2 mutations aid in the diagnosis of oligodendrogliomas (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 | 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 |
| Clinical Trial | Phase | Therapies | Title | Recruitment Status | Covered Countries | Other Countries |
|---|---|---|---|---|---|---|
| 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 | Not yet recruiting | ||
| NCT02968940 | Phase II | Avelumab | Avelumab With Hypofractionated Radiation Therapy in Adults With Isocitrate Dehydrogenase (IDH) Mutant Glioblastoma | Completed | ||
| NCT02525692 | Phase II | ONC201 | Oral ONC201 in Recurrent GBM, H3 K27M Glioma, and Midline Glioma | Recruiting | ||
| NCT03557359 | Phase II | Nivolumab | Nivolumab for Recurrent or Progressive IDH Mutant Gliomas | Recruiting | ||
| 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 | ||
| NCT04764474 | Phase I | HMPL-306 | A Study of HMPL-306 in Advanced Hematological Malignancies With mIDH | Not yet recruiting | ||
| NCT02576444 | Phase II | Adavosertib + Olaparib Olaparib Capivasertib + Olaparib AZD6738 + Olaparib | OLAParib COmbinations (OLAPCO) | Active, not recruiting | ||
| NCT04203316 | Phase II | Enasidenib | Enasidenib for the Treatment of Relapsed or Refractory Acute Myeloid Leukemia Patients With an IDH2 Mutation | Recruiting | ||
| NCT03683433 | Phase II | Azacitidine + Enasidenib | Enasidenib and Azacitidine in Treating Patients With Recurrent or Refractory Acute Myeloid Leukemia and IDH2 Gene Mutation | Recruiting | ||
| NCT04629209 | Phase II | ONC201 | A Phase II, Open Label Study of ONC201 in Adults With EGFR-low Glioblastoma | Not yet recruiting | ||
| 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 | ||
| 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 | ||
| NCT02428855 | Phase II | Dasatinib | Phase II Trial of Dasatinib in Patients With Isocitrate Dehydrogenase (IDH)-Mutant Advanced Intrahepatic Cholangiocarcinoma | Completed | ||
| NCT04118036 | Phase II | Abemaciclib + Pembrolizumab | Abemaciclib + Pembrolizumab In Glioblastoma | Suspended | ||
| NCT04015700 | Phase I | GNOS-PV01 + INO-9012 | Neoantigen-based Personalized DNA Vaccine in Patients With Newly Diagnosed, Unmethylated Glioblastoma | Recruiting | ||
| NCT03718767 | Phase II | Nivolumab | Nivolumab in Patients With IDH-Mutant Gliomas With and Without Hypermutator Phenotype | Recruiting | ||
| NCT03212274 | Phase II | Olaparib | Olaparib in Treating Patients With Advanced Glioma, Cholangiocarcinoma, or Solid Tumors With IDH1 or IDH2 Mutations | Recruiting | ||
| 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 | ||
| NCT03430791 | Phase II | Ipilimumab + Nivolumab Nivolumab | Trial of Combination TTF(Optune), Nivolumab Plus/Minus Ipilimumab for Recurrent Glioblastoma | Terminated | ||
| NCT03922555 | Phase I | Decitabine and Cedazuridine | ASTX727 in Recurrent/Progressive Non-enhancing IDH Mutant Gliomas | Recruiting | ||
| NCT03749187 | Phase I | Pamiparib + Temozolomide | BGB-290 and Temozolomide in Treating Isocitrate Dehydrogenase (IDH)1/2-Mutant Grade I-IV Gliomas (PNOC017) | Recruiting | ||
| NCT03047993 | Phase Ib/II | Azacitidine + Telaglenastat | Glutaminase Inhibitor CB-839 and Azacitidine in Treating Patients With Advanced Myelodysplastic Syndrome | Recruiting | ||
| 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 | ||
| NCT02981940 | Phase II | Abemaciclib | A Study of Abemaciclib in Recurrent Glioblastoma | Active, not recruiting | ||
| NCT02858895 | Phase II | MDNA55 | Convection-Enhanced Delivery (CED) of MDNA55 in Adults With Recurrent or Progressive Glioblastoma | Completed | ||
| NCT04606316 | Phase I | Nivolumab Ipilimumab + Nivolumab | Surgical Nivolumab And Ipilimumab For Recurrent GBM | Recruiting | ||
| NCT03728335 | Phase I | Enasidenib | Enasidenib as Maintenance Therapy in Treating Patients With Acute Myeloid Leukemia With IDH2 Mutation After Donor Stem Cell Transplant | Recruiting | ||
| 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 | ||
| NCT01915498 | Phase I | Enasidenib | Phase 1/2 Study of AG-221 in Subjects With Advanced Hematologic Malignancies With an IDH2 Mutation | Active, not recruiting | ||
| 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 | ||
| 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 | ||
| 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 | ||
| NCT03825796 | Phase II | CPX-351 + Enasidenib Cytarabine + Enasidenib Enasidenib | CPX-351 Plus Enasidenib for Relapsed AML | Recruiting | ||
| NCT03991832 | Phase II | Durvalumab + Olaparib | Study of Olaparib and Durvalumab in IDH-Mutated Solid Tumors | Recruiting | ||
| NCT03881735 | Phase II | Enasidenib | Enasidenib in Treating Patients With Relapsed or Refractory Acute Myeloid Leukemia With an IDH2 Gene Mutation | Withdrawn | ||
| NCT03207347 | Phase II | Niraparib | A Trial of Niraparib in BAP1 and Other DNA Damage Response (DDR) Deficient Neoplasms (UF-STO-ETI-001) | Recruiting | ||
| NCT04762602 | Phase I | HMPL-306 | A Study of HMPL-306 in Advanced Solid Tumors With IDH Mutations | Not yet recruiting | ||
| NCT03878095 | Phase II | AZD6738 + Olaparib | Testing Olaparib and AZD6738 in IDH1 and IDH2 Mutant Tumors | Recruiting | ||
| NCT03139916 | Phase II | Bavituximab + Temozolomide | Bavituximab With Radiation and Temozolomide for Patients With Newly Diagnosed Glioblastoma | Active, not recruiting | ||
| 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 | ||
| 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 | Not yet recruiting |