Revumenib (menin inhibitor) FDA-approved (Nov 2024) for relapsed/refractory KMT2A-rearran...
Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.
| ID | BMA-KMT2A-REARR-B-ALL |
|---|---|
| Type | Actionability |
| Status | reviewed 2026-05-03 | pending_clinical_signoff | actionability review required |
| Diseases | DIS-B-ALL |
| Sources | SRC-NCCN-ALL-2025 |
Actionability Facts
| Biomarker | BIO-KMT2A-REARRANGEMENT |
|---|---|
| Variant | KMT2A (MLL) rearrangement — any fusion partner; infant ALL (KMT2A-AFF1/AF4 most common ~80%) or adult B-ALL |
| Disease | DIS-B-ALL |
| ESCAT tier | IA |
| Recommended combinations | 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) |
| Evidence summary | 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. |
Notes
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.
Used By
No reverse references found in the YAML corpus.