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IDH2 R140Q or R172K activating mutation in AML — ~8-12% prevalence. Enasidenib (IDHIFA /...

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IDRF-AML-IDH2-MUT-ACTIONABLE
TypeSignal d’alerte
Étatreviewed 2026-04-27 | pending_clinical_signoff
MaladiesDIS-AML
SourcesSRC-ELN-AML-2022 SRC-ESMO-AML-2020 SRC-NCCN-AML-2025

Origine du drapeau rouge

DéfinitionIDH2 R140Q or R172K activating mutation in AML — ~8-12% prevalence. Enasidenib (IDHIFA / IDHENTIFY — phase-3 R/R AML; monotherapy ORR 38%, CR 19%) is FDA-approved for R/R-AML-IDH2; also active 1L combination with azacitidine in unfit (AG221-AML-005 — ORR 71%).
Direction cliniqueintensifier
Catégoriehigh-risk-biology
Algorithme de décalageALGO-AML-1L, ALGO-AML-2L

Logique de déclenchement

{
  "any_of": [
    {
      "finding": "idh2_r140q",
      "value": true
    },
    {
      "finding": "idh2_r172k",
      "value": true
    },
    {
      "finding": "idh2_mutation",
      "value": true
    },
    {
      "finding": "idh2_status",
      "value": "mutated"
    },
    {
      "finding": "idh_mutation",
      "value": "IDH2"
    }
  ],
  "type": "biomarker"
}

Notes

Detection: NGS panel (no IDH2-specific IHC widely deployed). Differentiation syndrome on enasidenib (~14%) — same monitoring + dexamethasone protocol as ivosidenib. Hyperbilirubinemia on enasidenib (~80% any grade; UGT1A1 inhibition — usually asymptomatic, no dose adjustment unless symptomatic). IDHENTIFY R/R AML did not show OS benefit vs conventional care over all comers but enriched in IDH2-mut who had not received prior hypomethylating agent. Combination with venetoclax + azacitidine under study.

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Algorithmes

Biomarqueur

Indication