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    GENE:

    IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2)

    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
    1d
    Rare Gain-of-Function Variants in IDH1 and IDH2 Confer Increased Risk of Immune-Mediated Rheumatic Diseases. (PubMed, ACR Open Rheumatol)
    Rare gain-of-function variants in IDH1/IDH2 are associated with increased risk of immune-mediated rheumatic diseases, particularly rheumatoid arthritis and polymyalgia rheumatica.
    Journal
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    IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2)
    5d
    Isocitrate Dehydrogenase-Mutant Astrocytomas: Risk Stratification and Therapeutic Advance. (PubMed, MedComm (2020))
    Therapeutic advances are highlighted, with particular emphasis on brain-penetrant IDH inhibition (vorasidenib) and on emerging strategies including vaccines, checkpoint combinations, epigenetic modulation, metabolic and microenvironment targeting, and novel delivery platforms...Finally, future directions in trial design, survivorship-oriented endpoints, and biomarker-driven monitoring are outlined. A trajectory-based paradigm is emphasized in which neurocognitive preservation, time to radiotherapy or chemotherapy, and patient-reported outcomes are prioritized while durable disease control is pursued across decades-long survivorship.
    Review • Journal
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    TP53 (Tumor protein P53) • IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2) • CDKN2A (Cyclin Dependent Kinase Inhibitor 2A) • CDKN2B (Cyclin Dependent Kinase Inhibitor 2B) • ATRX (ATRX Chromatin Remodeler)
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    CDKN2A deletion
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    Voranigo (vorasidenib)
    5d
    Acid Ceramidase Inhibition Disrupts Ceramide Homeostasis and Induces Mitochondrial Apoptosis in IDH1-Mutant Oligodendroglioma. (PubMed, Res Sq)
    These mechanistic effects translated into a survival benefit in oligodendroglioma xenograft-bearing mice. Together, these findings suggest that IDH1-mutant oligodendroglioma harbors a pre-existing heightened sensitivity to ceramide stress and identify acid ceramidase as a therapeutically actionable target in this disease.
    Journal
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    IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2)
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    IDH1 mutation
    6d
    Efficacy and Safety of Lisafotoclax Plus Decitabine and Homoharringtonine in Venetoclax/Azacitidine Pretreated AML Patients (clinicaltrials.gov)
    P2, N=35, Not yet recruiting, Second Affiliated Hospital, School of Medicine, Zhejiang University
    New P2 trial
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    FLT3 (Fms-related tyrosine kinase 3) • IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2) • NPM1 (Nucleophosmin 1)
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    FLT3-ITD mutation • NPM1 mutation
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    Venclexta (venetoclax) • azacitidine • decitabine • Synribo (omacetaxine mepesuccinate)
    6d
    Multidisciplinary consensus recommendations for the management of IDH-mutant grade 2 gliomas in Spain: a Delphi study. (PubMed, Clin Transl Oncol)
    This work delivers multidisciplinary consensus recommendations to standardize care in IDH-mutant grade 2 gliomas, integrating advances in molecular diagnostics, imaging, and therapeutics. It also identifies key knowledge gaps and clinical uncertainties, underscoring the critical need for ongoing research and expert collaboration to continually refine personalized management approaches and improve patient outcomes.
    Journal
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    IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2)
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    Voranigo (vorasidenib)
    7d
    Circulating tumor DNA-based detection of molecular residual disease in isocitrate dehydrogenase-mutant cholangiocarcinoma for biomarker-guided therapy. (PubMed, Clin Transl Oncol)
    Current first-line therapy for advanced IDH1-mutant disease includes gemcitabine plus cisplatin combined with either durvalumab or pembrolizumab. Ivosidenib is commonly used as a second-line targeted therapy...More prospective clinical trials are needed to validate ctDNA-guided risk stratification, support treatment decisions on adjuvant therapy escalation or de-escalation, and enable treatment adaptation in IDH-mutant CCA, ultimately advancing precision oncology beyond the capabilities of imaging alone. This review aims to evaluate the potential of ctDNA-based MRD detection to refine precision treatment strategies in IDH-mutant CCA.
    Review • Journal • PD(L)-1 Biomarker • Circulating tumor DNA
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    IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2)
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    IDH1 mutation
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    Keytruda (pembrolizumab) • cisplatin • Imfinzi (durvalumab) • gemcitabine • Tibsovo (ivosidenib)
    7d
    Transforming Acute Myeloid Leukemia Care Through Precision Medicine: Integrating Genomic Profiling, Measurable Residual Disease, and Targeted Therapies. (PubMed, Oncology)
    Measurable residual disease (MRD) assessment using multiparameter flow cytometry and molecular assays is increasingly used to refine post-remission decisions, including transplantation and maintenance strategies, but is constrained by assay variability, clonal hematopoiesis, and limited evidence for MRD-directed interventions. This narrative review synthesizes the evolving genomic landscape, guideline-endorsed targeted therapies, and investigational strategies in AML, and provides a practical framework that integrates genomic profiling, MRD-informed decision-making, and algorithmic treatment pathways while highlighting real-world barriers to implementation and equity.
    Review • Journal
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    FLT3 (Fms-related tyrosine kinase 3) • IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2) • NPM1 (Nucleophosmin 1)
    7d
    Presence of IDH2 and TP53 mutations significantly reduces survival of patients with chondrosarcoma. (PubMed, Cancer)
    IDH2 and TP53 mutations are enriched in dedifferentiated CS and are significant, independent predictors of adverse survival, regardless of tumor grade. These findings support IDH2 mutation status as clinically meaningful prognostic biomarker that may allow risk stratification and clinical decision-making in CS patients.
    Retrospective data • Journal
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    TP53 (Tumor protein P53) • IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2) • CDKN2A (Cyclin Dependent Kinase Inhibitor 2A) • TERT (Telomerase Reverse Transcriptase) • CDKN2B (Cyclin Dependent Kinase Inhibitor 2B)
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    TP53 mutation • IDH1 mutation • IDH2 mutation • IDH wild-type • IDH1 R132 • IDH2 R172
    8d
    Digital PCR of CHIP and MR for MRD Monitoring After Allo-HSCT in AML (clinicaltrials.gov)
    P=N/A, N=100, Recruiting, Peking University People's Hospital
    New trial • Tumor mutational burden • Minimal residual disease
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    ABL1 (ABL proto-oncogene 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2) • NPM1 (Nucleophosmin 1) • JAK2 (Janus kinase 2) • RUNX1 (RUNX Family Transcription Factor 1) • SF3B1 (Splicing Factor 3b Subunit 1) • ASXL1 (ASXL Transcriptional Regulator 1) • KMT2A (Lysine Methyltransferase 2A) • TET2 (Tet Methylcytosine Dioxygenase 2) • SRSF2 (Serine and arginine rich splicing factor 2) • BCOR (BCL6 Corepressor) • NUP98 (Nucleoporin 98 And 96 Precursor 2) • U2AF1 (U2 Small Nuclear RNA Auxiliary Factor 1) • STAG2 (Stromal Antigen 2) • NSD1 (Nuclear Receptor Binding SET Domain Protein 1) • HOXA9 (Homeobox A9) • NUP214 (Nucleoporin 214) • FUS (FUS RNA Binding Protein) • ZRSR2 (Zinc Finger CCCH-Type, RNA Binding Motif And Serine/Arginine Rich 2)
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    NPM1 mutation • MLL rearrangement
    10d
    Reconsidering time from diagnosis to treatment in acute myeloid leukemia: from therapeutic urgency to biologically informed timing. (PubMed, Expert Rev Hematol)
    Large retrospective and real-world studies suggest that short, biologically motivated deferrals aimed at completing molecular diagnostics do not compromise survival, including in venetoclax-based regimens, whereas prolonged system-related delays remain detrimental...In the NGS era, the diagnostic-to-treatment window should be regarded as an active, biologically informed phase of care rather than a passive delay. TDT should be viewed as a biology-adapted decision rather than an urgency metric: short, purposeful delays to complete genomic profiling can optimize treatment selection without harming outcomes, whereas system-driven delays remain detrimental and inequitable.
    Review • Journal
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    TP53 (Tumor protein P53) • IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2)
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    TP53 mutation
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    Venclexta (venetoclax)
    10d
    A Study of Gilteritinib in Combination With Ivosidenib or Enasidenib in People With Acute Myeloid Leukemia (AML) (clinicaltrials.gov)
    P1, N=18, Recruiting, Memorial Sloan Kettering Cancer Center | N=36 --> 18
    Enrollment change
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    FLT3 (Fms-related tyrosine kinase 3) • IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2)
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    IDH1 mutation • IDH2 mutation • FLT3 mutation
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    Xospata (gilteritinib) • Tibsovo (ivosidenib) • Idhifa (enasidenib)
    11d
    Integrating Targeted Therapies into AML Frontline Therapy: Who Gets What and What Does the Future Hold? (PubMed, Cancers (Basel))
    A combination that has become standard of care is HMA plus venetoclax for patients unfit for intensive chemotherapy, achieving high remission rates with relatively manageable toxicity...Clinical decision-making requires rapid molecular diagnostic testing, assessment of a patient's fitness for intensive chemotherapy, and management of toxicities and drug interactions. This narrative review, illustrated with patient vignettes, summarizes currently available therapies, guides through the latest trials on frontline combinations in AML, and provides a preview of how the therapeutic landscape may evolve in the near future.
    Review • Journal • IO biomarker
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    IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • BCL2 (B-cell CLL/lymphoma 2) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2) • CD33 (CD33 Molecule)
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    Venclexta (venetoclax)