Universität zu Köln

Braun, Till, Dechow, Annika, Friedrich, Gregor, Seifert, Michael, Stachelscheid, Johanna ORCID: 0000-0001-7419-8900 and Herling, Marco (2021). Advanced Pathogenetic Concepts in T-Cell Prolymphocytic Leukemia and Their Translational Impact. Front. Oncol., 11. LAUSANNE: FRONTIERS MEDIA SA. ISSN 2234-943X

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Abstract

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.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Braun, Till UNSPECIFIED UNSPECIFIED UNSPECIFIED Dechow, Annika UNSPECIFIED UNSPECIFIED UNSPECIFIED Friedrich, Gregor UNSPECIFIED UNSPECIFIED UNSPECIFIED Seifert, Michael UNSPECIFIED UNSPECIFIED UNSPECIFIED Stachelscheid, Johanna UNSPECIFIED orcid.org/0000-0001-7419-8900 UNSPECIFIED Herling, Marco UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-581088
DOI:	10.3389/fonc.2021.775363
Journal or Publication Title:	Front. Oncol.
Volume:	11
Date:	2021
Publisher:	FRONTIERS MEDIA SA
Place of Publication:	LAUSANNE
ISSN:	2234-943X
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language CHROMOSOMAL IMBALANCES; ATM GENE; EXPRESSION; MUTATIONS; ABNORMALITIES; PATHWAY Multiple languages Oncology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/58108