KMT2A rearrangement (MLL fusion at 11q23) in AML — ~5-10% prevalence in adult AML; ELN-20...
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| ID | RF-AML-KMT2A-ACTIONABLE |
|---|---|
| Tipo | Sinal de alerta |
| Estado | reviewed 2026-04-30 | pending_clinical_signoff |
| Doenças | DIS-AML |
| Fontes | SRC-ELN-AML-2022 SRC-NCCN-AML-2025 |
Origem da bandeira vermelha
| Definição | KMT2A rearrangement (MLL fusion at 11q23) in AML — ~5-10% prevalence in adult AML; ELN-2022 adverse-risk classifier. Revumenib (menin inhibitor; AUGMENT-101 phase 1/2) FDA-approved Nov 2024 for relapsed/ refractory KMT2A-rearranged acute leukemia after ≥1 prior line (ORR ~63%, CR/CRh ~23%). Ziftomenib alternative menin inhibitor in late-phase trials. Common partners: AF9 (t(9;11)) and AF4 (t(4;11)). |
|---|---|
| Direcção clínica | intensificar |
| Categoria | high-risk-biology |
| Algoritmo de deslocamentos | ALGO-AML-1L, ALGO-AML-2L |
Lógica de Desencadeamento
{
"any_of": [
{
"finding": "kmt2a_rearrangement",
"value": true
},
{
"finding": "kmt2a_fusion",
"value": true
},
{
"finding": "mll_rearrangement",
"value": true
},
{
"finding": "mll_fusion",
"value": true
},
{
"finding": "kmt2a_status",
"value": "rearranged"
},
{
"finding": "karyotype_11q23",
"value": true
}
],
"type": "biomarker"
}
Notas
Detection: karyotype + FISH break-apart probe at diagnosis; RT-PCR or RNA-NGS for fusion partner identification. Differentiation syndrome on revumenib (~16%) — monitor for fever, dyspnea, weight gain, effusions; treat with dexamethasone + hold revumenib until resolution. QTc prolongation requires baseline + serial ECG with K+/Mg++ correction. AlloHCT bridge for fit responders — revumenib alone NOT curative. Algorithm wiring deferred — IND-AML-2L-KMT2A- REVUMENIB created but not yet routed by ALGO-AML-2L (would require step-restructure, out of chunk scope). MDT review during chunk rollout. STUB — requires clinical co-lead signoff.
Usado por
Algoritmos
ALGO-AML-2L- ALGO-AML-2L
Indicação
IND-AML-2L-KMT2A-REVUMENIB- IND-AML-2L-KMT2A-REVUMENIB