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EZH2-driven immune evasion defines high-risk pediatric AML with t(16;21) FUS::ERG gene fusion.

Nathaniel J ButeynConnor G BurkeVincent J SartoriEve Deering-GardnerZachary J DeBruineDahlya KamarudinDarrell P ChandlerAlexander C MonovichMonika W PerezJoanna S YiRhonda E RiesTodd A AlonzoRussell J H RyanSoheil MeshinchiTimothy J Triche
Published in: bioRxiv : the preprint server for biology (2024)
pAML patients have dismal outcomes. Here we show a ubiquitous immune-evasive phenotype, defined by elevated EZH2 levels and loss of MHC class I and II receptors, is present in these patients at diagnosis. Treatment with the EZH2 inhibitor Tazemetostat successfully reverses the phenotype in a patient-derived cell line model.
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