These findings expand the genotypic spectrum of NBS and demonstrate that compound heterozygosity for non-classical NBN variants can lead to an adult identification of the disease. These cases highlight the importance of integrating genetic and functional analyses to facilitate the recognition of DNA repair disorders beyond childhood and to guide appropriate clinical surveillance and management.
Despite CHOP, CHOEP, DA-EPOCH, and AC-CHOP (azacitidine plus chidamide) regimens, the patient experienced primary refractory disease and died 6 months from diagnosis following COVID-19 superinfection. To our knowledge, this is the first case reporting single-cell transcriptomic comparison of skin versus blood compartments in PTCL-NOS, revealing how cutaneous microenvironment sculpts aggressive malignant phenotypes and providing potential targets for compartment-specific therapy.
These findings show improved outcomes in early stage nodal T-cell lymphomas compared to historical data for allcomers. In addition, favorable outcomes in stage I disease treated with chemoradiation support such strategy as an excellent treatment option in this setting.
In PMBCL, the Children's Oncology Group (COG) and the National Cancer Institute (NCI) National Clinical Trials Network (NCTN) recently completed a randomized phase III trial of nivolumab in combination with chemo-immunotherapy in children and adults with newly diagnosed PMBCL...In ALCL and ANKL, ongoing clinical trials are evaluating the efficacy of ICT. Given the transformational role of ICT for pediatric Hodgkin lymphoma, there is significant promise for the use of ICT in multiple subtypes of pediatric non-Hodgkin lymphoma with increased expression of PD-L1.