Molecular Profile Detail

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Profile Name IDH1 R132X
Gene Variant Detail

IDH1 R132X (unknown)

Relevant Treatment Approaches IDH Inhibitor (Pan) IDH1 Inhibitor

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Molecular Profile Indication/Tumor Type Response Type Relevant Treatment Approaches Therapy Name Approval Status Evidence Type Efficacy Evidence References
IDH1 R132X acute myeloid leukemia no benefit IDH1 Inhibitor BAY1436032 Phase I Actionable In a Phase I trial, treatment with BAY1436032 in acute myeloid leukemia patients harboring an IDH1 R132X mutation demonstrated safety and resulted in an overall response rate of 15% (4/27), including one complete remission, one partial remission, and morphologic leukemia-free state in two patients, stable disease in 67% (18/27), and a median overall survival of 6.6 months, however, due to low response rates for all doses, further clinical development was not supported (PMID: 32733012; NCT03127735). 32733012
IDH1 R132X acute myeloid leukemia sensitive IDH1 Inhibitor Azacitidine + Ivosidenib Phase III Actionable In a Phase III trial (AGILE), Tibsovo (ivosidenib) and Vidaza (azacytidine) combination therapy significantly improved event-free survival (HR 0.33, p=0.002) and median overall survival (24.0 vs 7.9 mo, HR 0.44, p=0.001) compared to Vidaza (azacytidine) plus placebo in patients with newly diagnosed acute myeloid leukemia harboring IDH1 mutations including R132C/H/G/L/S (PMID: 35443108; NCT03173248). 35443108
IDH1 R132X acute myeloid leukemia predicted - sensitive CG-806 Preclinical - Patient cell culture Actionable In a preclinical study, patient-derived acute myeloid leukemia cells harboring IDH1 R132X mutations demonstrated increased sensitivity to CG-806 compared to wild-type cells in culture (Proceedings of the American Association for Cancer Research, Vol 60, Mar 2019, Abstract #1323). detail...
IDH1 R132X acute myeloid leukemia sensitive IDH1 Inhibitor IDH305 Phase I Actionable In a Phase I trial, IDH305 treatment resulted in objective response in 33% (7/21) of acute myeloid leukemia patients harboring IDH1 R132 mutations, including complete remission in 3 (14%) and partial remission in 4 (19%) patients (Blood 2016 128 (22):1073). detail...
IDH1 R132X low grade glioma predicted - sensitive IDH1 Inhibitor Ivosidenib Phase I Actionable In a Phase I trial, low grade glioma patients with an IDH1 mutation (n=66; R132H=57, R132C/G/S=1 each, R132X=5) treated with Tibsovo (ivosidenib) demonstrated an overall response rate of 2.9% (1/35, 1 partial response) and stable disease in 85.7% (30/35) of patients with a non-enhancing glioma versus no responses and stable disease in 45.2% (14/31) of patients with an enhancing glioma, and led to a median progression-free survival of 13.6 months and 1.4 months, respectively (PMID: 32530764; NCT02073994). 32530764
Clinical Trial Phase Therapies Title Recruitment Status Covered Countries Other Countries
NCT02381886 Phase I IDH305 A Study of IDH305 in Patients With Advanced Malignancies That Harbor IDH1R132 Mutations Active, not recruiting USA | ESP | DEU | CAN | BEL 3
NCT03839771 Phase III Ivosidenib Enasidenib A Study of Ivosidenib or Enasidenib in Combination With Induction Therapy and Consolidation Therapy, Followed by Maintenance Therapy in Patients With Newly Diagnosed Acute Myeloid Leukemia or Myedysplastic Syndrome EB2, With an IDH1 or IDH2 Mutation, Respectively, Eligible for Intensive Chemotherapy (HOVON150AML) Recruiting FRA | ESP | DEU | BEL | AUT 10
NCT04521686 Phase I LY3410738 Study of LY3410738 Administered to Patients With Advanced Solid Tumors With IDH1 or IDH2 Mutations Recruiting USA | FRA | ESP 6
NCT02977689 Phase II IDH305 Trial of IDH305 in IDH1 Mutant Grade II or III Glioma Withdrawn USA 0
NCT04603001 Phase I LY3410738 Study of Oral LY3410738 in Patients With Advanced Hematologic Malignancies With IDH1 or IDH2 Mutations Recruiting USA | FRA | ESP | DEU | CAN | BEL 6
NCT03684811 Phase Ib/II Cisplatin + Gemcitabine + Olutasidenib Azacitidine + Olutasidenib Olutasidenib Nivolumab + Olutasidenib A Study of FT 2102 in Participants With Advanced Solid Tumors and Gliomas With an IDH1 Mutation Active, not recruiting USA | FRA | ESP 3
NCT04250051 Phase I Cytarabine + Filgrastim + Fludarabine + Ivosidenib Ivosidenib Ivosidenib and Combination Chemotherapy for the Treatment of IDH1 Mutant Relapsed or Refractory Acute Myeloid Leukemia Not yet recruiting USA 0
NCT03528642 Phase I Radiotherapy + Telaglenastat + Temozolomide Telaglenastat With Radiation Therapy and Temozolomide in Treating Patients With IDH-Mutated Diffuse Astrocytoma or Anaplastic Astrocytoma Active, not recruiting USA 0
NCT03564821 Phase I Ivosidenib IDH1 Inhibition Using Ivosidenib as Maintenance Therapy for IDH1-mutant Myeloid Neoplasms Following Allogeneic Stem Cell Transplantation Active, not recruiting USA 0
NCT04013880 Phase Ib/II Decitabine and Cedazuridine + Olutasidenib ASTX727 and FT-2102 in Treating IDH1-Mutated Recurrent/Refractory Myelodysplastic Syndrome or Acute Myeloid Leukemia Withdrawn 0
NCT04493164 Phase II CPX-351 + Ivosidenib Ivosidenib CPX-351 and Ivosidenib for the Treatment of IDH1 Mutated Acute Myeloid Leukemia or High-Risk Myelodysplastic Syndrome Recruiting USA 0
NCT02746081 Phase I BAY1436032 Phase I Study of BAY1436032 in IDH1-mutant Advanced Solid Tumors Active, not recruiting USA | DEU 2
NCT02719574 Phase I Cytarabine + Olutasidenib Olutasidenib Azacitidine + Olutasidenib Open-label Study of FT-2102 With or Without Azacitidine or Cytarabine in Patients With AML or MDS With an IDH1 Mutation Active, not recruiting USA | ITA | FRA | ESP | DEU | CAN 3
NCT03471260 Phase Ib/II Azacitidine + Ivosidenib + Venetoclax Ivosidenib + Venetoclax Ivosidenib and Venetoclax With or Without Azacitidine in Treating Participants With IDH1 Mutated Hematologic Malignancies Recruiting USA 0
NCT02074839 Phase I Ivosidenib Study of Orally Administered AG-120 in Subjects With Advanced Hematologic Malignancies With an IDH1 Mutation Recruiting USA | FRA 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 | ITA | FRA | ESP | DEU | CAN 6
NCT03914742 Phase Ib/II Pamiparib + Temozolomide BGB-290 and Temozolomide in Treating Patients With Recurrent Gliomas With IDH1/2 Mutations Active, not recruiting USA 0
NCT04655391 Phase I Glasdegib Glasdegib + Ivosidenib Bosutinib + Glasdegib Gilteritinib + Glasdegib Decitabine + Glasdegib + Venetoclax Enasidenib + Glasdegib Glasdegib-Based Treatment Combinations for the Treatment of Patients With Relapsed Acute Myeloid Leukemia Who Have Undergone Hematopoietic Cell Transplantation Not yet recruiting USA 0