Braun, Till
[Verfasser:in];
Dechow, Annika
[Verfasser:in];
Friedrich, Gregor
[Verfasser:in];
Seifert, Michael
[Verfasser:in];
Stachelscheid, Johanna
[Verfasser:in];
Herling, Marco
[Verfasser:in]
Advanced Pathogenetic Concepts in T-Cell Prolymphocytic Leukemia and Their Translational Impact
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Medientyp:
E-Artikel
Titel:
Advanced Pathogenetic Concepts in T-Cell Prolymphocytic Leukemia and Their Translational Impact
Beteiligte:
Braun, Till
[Verfasser:in];
Dechow, Annika
[Verfasser:in];
Friedrich, Gregor
[Verfasser:in];
Seifert, Michael
[Verfasser:in];
Stachelscheid, Johanna
[Verfasser:in];
Herling, Marco
[Verfasser:in]
Erschienen:
Lausanne: Frontiers Research Foundation, [2023]
Beschreibung:
T-cell prolymphocytic leukemia (T-PLL) is the most common mature T-cell leukemia. It is atypically aggressively growing and chemotherapy-resistant malignancy with a poorprognosis. T-PLL cells resemble activated, post-thymic T-lymphocytes with memorytypeeffector functions. Constitutive transcriptional activation of genes of the T-cellleukemia 1 (TCL1) family based on genomic inversions/translocations is recognized asa key event in T-PLL’s pathogenesis. TCL1’s multiple effector pathways include theenhancement of T-cell receptor (TCR) signals. New molecular dependencies aroundresponses to DNA damage, including repair and apoptosis regulation, as well asalterations of cytokine and non-TCR activation signaling were identified as perturbedhallmark pathways within the past years. We currently witness these vulnerabilities to beinterrogated in first pre-clinical concepts and initial clinical testing in relapsed/refractory TPLLpatients. We summarize here the current knowledge on the molecular understandingof T-PLL’s pathobiology and critically assess the true translational progress around this tohelp appraisal by caregivers and patients. Overall, the contemporary concepts on T-PLL’spathobiology are condensed in a comprehensive mechanistic disease model andpromising interventional strategies derived from it are highlighted.