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    BIOMARKER:

    IDH2 mutation

    i
    Other names: IDH2, Isocitrate Dehydrogenase (NADP(+)) 2, Isocitrate Dehydrogenase (NADP(+)) 2, Mitochondrial, Isocitrate Dehydrogenase 2 (NADP+), Mitochondrial, Isocitrate Dehydrogenase [NADP], Mitochondrial, Oxalosuccinate Decarboxylase, NADP(+)-Specific ICDH, ICD-M, IDH, IDP, MNADP-IDH, D2HGA2, IDHM, IDPM
    Entrez ID:
    3418
    Related biomarkers:
    Expression
    Mutation
    Others
    Related tests:
    7d
    LS1781: Ascorbic Acid and Chemotherapy for the Treatment of Relapsed or Refractory Lymphoma, CCUS, and Chronic Myelomonocytic Leukemia (clinicaltrials.gov)
    P2, N=80, Recruiting, Mayo Clinic | Trial completion date: Mar 2027 --> Nov 2033 | Trial primary completion date: Mar 2027 --> Feb 2031
    Trial completion date • Trial primary completion date
    |
    IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • BCL2 (B-cell CLL/lymphoma 2) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2) • DNMT3A (DNA methyltransferase 1) • SF3B1 (Splicing Factor 3b Subunit 1) • TET2 (Tet Methylcytosine Dioxygenase 2) • SRSF2 (Serine and arginine rich splicing factor 2) • U2AF1 (U2 Small Nuclear RNA Auxiliary Factor 1) • CD4 (CD4 Molecule) • ZRSR2 (Zinc Finger CCCH-Type, RNA Binding Motif And Serine/Arginine Rich 2)
    |
    IDH2 mutation • TET2 mutation • SF3B1 mutation • EZH2 mutation • SRSF2 mutation
    |
    cisplatin • carboplatin • gemcitabine • Rituxan (rituximab) • cytarabine • cyclophosphamide • ifosfamide • oxaliplatin • etoposide IV • decitabine • Truxima (rituximab-abbs) • Hemady (dexamethasone tablets) • Mabtas (rituximab biosimilar) • Starasid (cytarabine ocfosfate) • dexamethasone injection
    9d
    GFH009X2101: Study of SLS009 (Formerly GFH009) a Potent Highly Selective CDK9 Inhibitor in Patients With Hematologic Malignancies and High-Risk Newly Diagnosed AML (clinicaltrials.gov)
    P1/2, N=160, Recruiting, Sellas Life Sciences Group | Trial completion date: Dec 2025 --> Dec 2027 | Trial primary completion date: Dec 2025 --> Dec 2026
    Trial completion date • Trial primary completion date
    |
    PD-L1 (Programmed death ligand 1) • KRAS (KRAS proto-oncogene GTPase) • FLT3 (Fms-related tyrosine kinase 3) • IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2) • NPM1 (Nucleophosmin 1) • SF3B1 (Splicing Factor 3b Subunit 1) • ASXL1 (ASXL Transcriptional Regulator 1) • SRSF2 (Serine and arginine rich splicing factor 2) • BCOR (BCL6 Corepressor) • U2AF1 (U2 Small Nuclear RNA Auxiliary Factor 1) • STAG2 (Stromal Antigen 2) • DDX41 (DEAD-Box Helicase 41) • ZRSR2 (Zinc Finger CCCH-Type, RNA Binding Motif And Serine/Arginine Rich 2)
    |
    TP53 mutation • KRAS mutation • FLT3-ITD mutation • Chr del(17p) • IDH1 mutation • IDH2 mutation • FLT3 mutation • TP53 wild-type • NPM1 mutation • KRAS wild-type • Chr del(11q) • ASXL1 mutation • SF3B1 mutation • EZH2 mutation • NRAS wild-type • SRSF2 mutation • IDH wild-type
    |
    Venclexta (venetoclax) • azacitidine • tambiciclib (SLS009)
    9d
    DNA Methylation Stochasticity is Linked to Transcriptional Variability and Convergent Epigenetic Disruption Across Genetic Subtypes of Acute Myeloid Leukemia. (PubMed, Cancer Res)
    Finally, the hypomethylating drug decitabine led to reduction of DNA methylation entropy specifically in IDH2-mutant AML cells. Overall, this approach identified a convergent program of epigenetic dysregulation in leukemia, clarifying the contribution of specific genetic mutations to stochastic disruption of the epigenetic and transcriptional landscapes of AML.
    Journal • Tumor mutational burden
    |
    TMB (Tumor Mutational Burden) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2) • CEBPA (CCAAT Enhancer Binding Protein Alpha)
    |
    IDH2 mutation • TMB-L
    |
    decitabine
    11d
    Enasidenib (AG-221) Maintenance Post Allogeneic HCT in Patients With IDH2 Mutation (clinicaltrials.gov)
    P2, N=35, Recruiting, City of Hope Medical Center | Suspended --> Recruiting
    Enrollment open
    |
    IDH2 mutation
    |
    Idhifa (enasidenib)
    12d
    IDH2 Clonal Hematopoiesis and IKAROS Loss Cooperate in a B-ALL Subtype after Lenalidomide Therapy for Multiple Myeloma. (PubMed, Blood)
    Subsequent genetic or epigenetic alterations render leukemogenesis independent of ongoing lenalidomide exposure. Altogether, these data define IDH2mt B-ALL as a distinct molecular subtype that is markedly overrepresented after lenalidomide treatment and highlight clonal hematopoiesis as a key contributing factor in the development of LenB-ALL.
    Journal
    |
    KRAS (KRAS proto-oncogene GTPase) • NRAS (Neuroblastoma RAS viral oncogene homolog) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2) • IKZF1 (IKAROS Family Zinc Finger 1)
    |
    TP53 mutation • IDH2 mutation
    |
    lenalidomide
    12d
    Multicenter evaluation of the Idylla IDH1-2 Mutation Assay Kit for detection of IDH mutational status in glioma. (PubMed, Virchows Arch)
    The validity of the Idylla IDH1-2 Mutation Assay was 98.06%. The Idylla IDH1-2 Mutation Assay Kit showed comparable performance to other well-validated methods for IDH mutation analysis while demonstrating excellent assay turnaround times.
    Journal
    |
    KIT (KIT proto-oncogene, receptor tyrosine kinase) • IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2)
    |
    IDH1 mutation • IDH2 mutation • KIT mutation • IDH wild-type
    |
    Idylla™ IDH1-2 Mutation Assay
    13d
    Acute myeloid leukaemia (AML) harbouring KMT2A-PTD: should it be considered as a myelodysplasia-related abnormality? (PubMed, J Clin Pathol)
    AML harbouring KMT2A-PTD frequently displays MR immunophenotypic abnormalities and is frequently associated with AML-MR type mutations. In addition, they have similar outcomes as compared with AML-MR.
    Journal
    |
    FLT3 (Fms-related tyrosine kinase 3) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2) • DNMT3A (DNA methyltransferase 1) • RUNX1 (RUNX Family Transcription Factor 1) • KMT2A (Lysine Methyltransferase 2A) • U2AF1 (U2 Small Nuclear RNA Auxiliary Factor 1) • CD7 (CD7 Molecule) • CD177 (CD177 Molecule)
    |
    IDH2 mutation • IDH2 R172
    14d
    Neoantigen-based Personalized DNA Vaccine With Retifanlimab PD-1 Blockade Therapy in Patients With Newly Diagnosed, Unmethylated Glioblastoma (clinicaltrials.gov)
    P1, N=27, Active, not recruiting, Washington University School of Medicine | Recruiting --> Active, not recruiting | Trial completion date: Jan 2030 --> Mar 2029 | Trial primary completion date: Apr 2027 --> Apr 2026
    Enrollment closed • Trial completion date • Trial primary completion date
    |
    IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2) • MGMT (6-O-methylguanine-DNA methyltransferase)
    |
    IDH2 mutation • MGMT promoter methylation • IDH wild-type
    |
    Zynyz (retifanlimab-dlwr) • neoantigen DNA vaccine
    16d
    Trial of Niraparib in Participants With Newly-diagnosed Glioblastoma and Recurrent Glioma (clinicaltrials.gov)
    P1, N=42, Active, not recruiting, Nader Sanai | Trial completion date: Sep 2025 --> Mar 2027
    Trial completion date
    |
    IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2) • ATRX (ATRX Chromatin Remodeler)
    |
    IDH2 mutation
    |
    Zejula (niraparib)
    16d
    Preliminary evidence for the integration of CNAs, CN-LOH and mutational profiles into the prognostic stratification of elderly patients with NPM1-mutated acute myeloid leukemia. (PubMed, Oncol Lett)
    These findings provide preliminary evidence that integrating CNA/CN-LOH burden with mutational profiles may improve risk stratification in elderly patients with NPM1-mutated AML. However, given the small sample size, single-center design and lack of external validation, larger multi-center studies are warranted to assess the robustness of these findings.
    Journal
    |
    FLT3 (Fms-related tyrosine kinase 3) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2) • NPM1 (Nucleophosmin 1) • DNMT3A (DNA methyltransferase 1) • TET2 (Tet Methylcytosine Dioxygenase 2)
    |
    IDH2 mutation • NPM1 mutation
    19d
    Enasidenib (AG-221) Maintenance Post Allogeneic HCT in Patients With IDH2 Mutation (clinicaltrials.gov)
    P2, N=35, Suspended, City of Hope Medical Center | Recruiting --> Suspended
    Trial suspension
    |
    IDH2 mutation
    |
    Idhifa (enasidenib)
    19d
    A Study of Gilteritinib in Combination With Ivosidenib or Enasidenib in People With Acute Myeloid Leukemia (AML) (clinicaltrials.gov)
    P1, N=36, Recruiting, Memorial Sloan Kettering Cancer Center | Trial completion date: Feb 2026 --> Feb 2027 | Trial primary completion date: Feb 2026 --> Feb 2027
    Trial completion date • Trial primary completion date
    |
    FLT3 (Fms-related tyrosine kinase 3) • IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2)
    |
    IDH1 mutation • IDH2 mutation • FLT3 mutation
    |
    Xospata (gilteritinib) • Tibsovo (ivosidenib) • Idhifa (enasidenib)