Advanced Pathogenetic Concepts in T-Cell Prolymphocytic Leukemia and Their Translational Impact.
In: Frontiers in Oncology, Jg. 11 (2021-11-19), S. 1-8
academicJournal
Zugriff:
T-cell prolymphocytic leukemia (T-PLL) is the most common mature T-cell leukemia. It is a typically aggressively growing and chemotherapy-resistant malignancy with a poor prognosis. T-PLL cells resemble activated, post-thymic T-lymphocytes with memory-type effector functions. Constitutive transcriptional activation of genes of the T-cell leukemia 1 (TCL1) family based on genomic inversions/translocations is recognized as a key event in T-PLL's pathogenesis. TCL1's multiple effector pathways include the enhancement of T-cell receptor (TCR) signals. New molecular dependencies around responses to DNA damage, including repair and apoptosis regulation, as well as alterations of cytokine and non-TCR activation signaling were identified as perturbed hallmark pathways within the past years. We currently witness these vulnerabilities to be interrogated in first pre-clinical concepts and initial clinical testing in relapsed/refractory T-PLL patients. We summarize here the current knowledge on the molecular understanding of T-PLL's pathobiology and critically assess the true translational progress around this to help appraisal by caregivers and patients. Overall, the contemporary concepts on T-PLL's pathobiology are condensed in a comprehensive mechanistic disease model and promising interventional strategies derived from it are highlighted. [ABSTRACT FROM AUTHOR]
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Advanced Pathogenetic Concepts in T-Cell Prolymphocytic Leukemia and Their Translational Impact.
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Autor/in / Beteiligte Person: | Braun, Till ; Dechow, Annika ; Friedrich, Gregor ; Seifert, Michael ; Stachelscheid, Johanna ; Herling, Marco |
Zeitschrift: | Frontiers in Oncology, Jg. 11 (2021-11-19), S. 1-8 |
Veröffentlichung: | 2021 |
Medientyp: | academicJournal |
ISSN: | 2234-943X (print) |
DOI: | 10.3389/fonc.2021.775363 |
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