Revumenib (menin inhibitor) FDA-approved (Nov 2024) for relapsed/refractory KMT2A-rearran...
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| ID | BMA-KMT2A-REARR-B-ALL |
|---|---|
| Tipo | Relevancia terapéutica |
| Situación | reviewed 2026-05-03 | pending_clinical_signoff | actionability review required |
| Enfermedades | DIS-B-ALL |
| Fuentes | SRC-NCCN-ALL-2025 |
Hechos relativos a la posibilidad de adoptar medidas
| Biomarcador | BIO-KMT2A-REARRANGEMENT |
|---|---|
| Variante | Rearreglo de KMT2A (MLL) — cualquier pareja de fusión; LLA infantil (KMT2a-AFF1/AF4 más común ~80%) o B-ALL adulto |
| Enfermedad | DIS-B-ALL |
| El nivel de ESCAT | IA |
| Combinaciones recomendadas | revumenib 163 mg PO BID monotherapy (dose-adjusted for CYP3A4 inhibitors to 95 mg BID), blinatumomab (independent of KMT2A status; CD19-targeted; R/R B-ALL per TOWER trial), inotuzumab ozogamicin (CD22-targeted; R/R B-ALL per INO-VATE trial) |
| Resumen de las pruebas | Revumenib (menin inhibitor) FDA-approved (Nov 2024) for relapsed/refractory KMT2A-rearranged acute leukemia including B-ALL. AUGMENT-101 combined KMT2A-rearranged cohort (AML + ALL): ORR 23%, CR/CRh 18%. KMT2A rearrangement defines a high-risk B-ALL subset: in infants KMT2A-AFF1 is present in ~80% of infant B-ALL (EFS <30% with standard chemotherapy); in adults KMT2A-rearranged B-ALL is adverse-risk (NCCN/ELN). Revumenib represents a first targeted option for this historically chemotherapy-refractory population. Blinatumomab (CD19 BiTE, FDA-approved for R/R B-ALL) and inotuzumab ozogamicin are also active in KMT2A-rearranged B-ALL regardless of KMT2A status, but revumenib is the first mutation-specific approved agent. AlloHCT in CR remains the standard consolidation for eligible adults. |
Notas
ESCAT IA (accelerated approval, confirmatory trial ongoing). KMT2A-AFF1 (t(4;11)) is the most common rearrangement in adult B-ALL and is uniformly adverse-risk per ELN/NCCN. Infant B-ALL with KMT2A rearrangement: revumenib use in pediatric/infant context is still experimental (AUGMENT-101 enrolled adults; pediatric trials underway). QTc monitoring and CYP3A4 interaction management apply equally to B-ALL as AML setting (see BMA-KMT2A-REARR-AML notes). AlloHCT in CR1 remains mandatory for all fit adults with KMT2A-rearranged B-ALL regardless of MRD status.
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