Lack of SMARCB1 expression characterizes a subset of human and murine peripheral T-cell lymphomas.

ABSTRACT: Peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) is a heterogeneous group of malignancies with poor outcome. Here, we identify a subgroup, PTCL-NOS^SMARCB1-, which is characterized by the lack of the SMARCB1 protein and occurs more frequently in young patients. Human and murine PTCL-NOS^SMARCB1- show similar DNA methylation profiles, with hypermethylation of T-cell-related genes and hypomethylation of genes involved in myeloid development. Single-cell analyses of human and murine tumors revealed a rich and complex network of interactions between tumor cells and an immunosuppressive and exhausted tumor microenvironment (TME). In a drug screen, we identified histone deacetylase inhibitors (HDACi) as a class of drugs effective against PTCL-NOS^Smarcb1-

SUBMITTER: Fischer A 

PROVIDER: S-EPMC11452211 | biostudies-literature | 2024 Oct

REPOSITORIES: biostudies-literature