OVERVIEW: Mixed lineage leukemia-rearranged (MLLr) and revumenib
Mixed lineage leukemia-rearranged (MLLr) is an acute leukemia with poor prognosis that is caused by spontaneous translocations at the MLL1 gene locus. MLLr fusion proteins bind with high affinity to the nuclear protein menin and the menin-MLL interaction enables leukemic transformation by driving a specific transcription program.1
In preclinical studies, inhibition of the Menin-MLL1 interaction led to tumor cell differentiation and death.1
CAP/ASH Testing Guidelines recommend the testing of MLL1 (KMT2A) and NPM1 mutations as part of the diagnostic workup of acute leukemia.1,2
Adapted from: Uckelmann HJ, et al. Presented at ASH Annual Meeting, 2018.
- Kuhn MW, Armstrong SA. Designed to kill: novel menin-MLL inhibitors target MLL-rearranged leukemia. Cancer Cell. 2015;27(4):431-433.
- Arber DA, Borowitz MJ, Cessna M, et al. Initial diagnostic workup of acute leukemia: guideline from the College of American Pathologists and the American Society of Hematology. Arch Pathol Lab Med. 2017;141(10):1342-1393.