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Profile Name | IDH2 R140X |
Gene Variant Detail | |
Relevant Treatment Approaches | IDH Inhibitor (Pan) IDH2 Inhibitor |
Molecular Profile | Indication/Tumor Type | Response Type | Relevant Treatment Approaches | Therapy Name | Approval Status | Evidence Type | Efficacy Evidence | References |
---|---|---|---|---|---|---|---|---|
IDH2 R140X | myelodysplastic syndrome | predicted - sensitive | IDH2 Inhibitor | Enasidenib | Phase II | Actionable | In a Phase II trial, Idhifa (enasidenib) treatment was well tolerated and resulted in an overall response rate of 43% (9/21, 5 complete remission (CR), 1 partial remission, 1 marrow CR, 2 hematological improvement only) in patients with higher-risk myelodysplastic syndrome harboring IDH2 R140 or R172 mutations who were refractory to or progressed on hypomethylating agents, with a median overall survival of 21.3 months (J Clin Oncol 39, no. 15_suppl (May 20, 2021) 7010-7010; NCT03383575). | detail... |
IDH2 R140X | myelodysplastic syndrome | predicted - sensitive | IDH2 Inhibitor | Azacitidine + Enasidenib | Phase II | Actionable | In a Phase II trial, Idhifa (enasidenib) and Vidaza (azacitidine) combination treatment was well tolerated and resulted in an overall response rate of 68% (30/46, 11 complete remission (CR), 3 partial remission, 12 marrow CR, 4 hematological improvement only) in patients with higher-risk myelodysplastic syndrome harboring IDH2 R140 or R172 mutations who were naive to hypomethylating agents, with a median overall survival of 21.3 months (J Clin Oncol 39, no. 15_suppl (May 20, 2021) 7010-7010; NCT03383575). | detail... |
IDH2 R140X | acute myeloid leukemia | predicted - sensitive | LY3410738 | Phase I | Actionable | In a Phase I trial, LY3410738 demonstrated safety and inhibited D-2-HG in patients with relapsed or refractory IDH-mutant acute myeloid leukemia, resulting in a composite complete remission (CRc) rate of 9% (2/23, 2 CR) in IDH inhibitor-naive patients harbor IDH2 R140 mutations (Cancer Res (2023) 83 (8_Supplement): CT026; NCT04603001). | detail... |