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IDH1 R132 (R132H/C/G/L/S) activating mutation in AML — ~6-10% prevalence. Ivosidenib (AGI...

Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.

IDRF-AML-IDH1-MUT-ACTIONABLE
TypeRed flag
Statusreviewed 2026-04-27 | pending_clinical_signoff
DiseasesDIS-AML
SourcesSRC-ELN-AML-2022 SRC-ESMO-AML-2020 SRC-NCCN-AML-2025

Red Flag Origin

DefinitionIDH1 R132 (R132H/C/G/L/S) activating mutation in AML — ~6-10% prevalence. Ivosidenib (AGILE — addition to azacitidine 1L unfit-for-intensive: mEFS HR 0.33, mOS 24.0 vs 7.9 mo) is FDA-approved for ND-AML-IDH1 unfit; 7+3 + ivosidenib in fit patients per emerging data. Monotherapy approved R/R-AML.
Clinical directionintensify
Categoryhigh-risk-biology
Shifts algorithmALGO-AML-1L, ALGO-AML-2L

Trigger Logic

{
  "any_of": [
    {
      "finding": "idh1_r132h",
      "value": true
    },
    {
      "finding": "idh1_mutation",
      "value": true
    },
    {
      "finding": "idh1_status",
      "value": "mutated"
    },
    {
      "finding": "idh_mutation",
      "value": "IDH1"
    }
  ],
  "type": "biomarker"
}

Notes

IHC (mIDH1-R132H antibody) is highly sensitive for the dominant R132H variant — used for first-pass screen. Other IDH1 variants (R132C, R132G, R132S, R132L) are rarer and IHC-negative — require NGS panel. Differentiation syndrome on ivosidenib (~25%) — monitor WBC, dyspnea, weight gain; dexamethasone treatment + hold IDH1i. AGILE trial: ivosidenib + azacitidine for unfit; 7+3 + ivosidenib emerging for fit (HOVON / NCCN-listed). Olutasidenib alternative (FDA-approved R/R-AML).

Used By

Algorithms

Indications