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- Actionable perturbations of damage responses by TCL1/ATM and epigenetic lesions form the basis of T-PLL
Actionable perturbations of damage responses by TCL1/ATM and epigenetic lesions form the basis of T-PLL
Auteurs
A. Schrader, G. Crispatzu, S. Oberbeck, P. Mayer, S. Pützer, J. von Jan, E. Vasyutina, K. Warner, N. Weit, N. Pflug, T. Braun, E. I. Andersson, B. Yadav, A. Riabinska, B. Maurer, M. S. Ventura Ferreira, F. Beier, J. Altmüller, M. Lanasa, C. D. Herling, T. Haferlach, S. Stilgenbauer, G. Hopfinger, M. Peifer, T. H. Brümmendorf, P. Nürnberg, K. S. J. Elenitoba-Johnson, S. Zha, M. Hallek, R. Moriggl, H. C. Reinhardt, M.-H. Stern, S. Mustjoki, S. Newrzela, P. Frommolt, M. Herling
Résumé
Abstract
T-cell prolymphocytic leukemia (T-PLL) is a rare and poor-prognostic mature T-cell malignancy. Here we integrated large-scale profiling data of alterations in gene expression, allelic copy number (CN), and nucleotide sequences in 111 well-characterized patients. Besides prominent signatures of T-cell activation and prevalent clonal variants, we also identify novel hot-spots for CN variability, fusion molecules, alternative transcripts, and progression-associated dynamics. The overall lesional spectrum of T-PLL is mainly annotated to axes of DNA damage responses, T-cell receptor/cytokine signaling, and histone modulation. We formulate a multi-dimensional model of T-PLL pathogenesis centered around a unique combination of
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