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@ARTICLE{Pfeuffer:298559,
author = {L. Pfeuffer and V. Siegert and R. Trozzo and K. Steiger$^*$
and R. Rad$^*$ and J. Ruland and M. Buchner},
title = {{A}utoimmunity promotes chronic lymphocytic leukemia
progression in an indolent disease model.},
journal = {Scientific reports},
volume = {15},
number = {1},
issn = {2045-2322},
address = {[London]},
publisher = {Springer Nature},
reportid = {DKFZ-2025-00284},
pages = {4117},
year = {2025},
abstract = {Chronic lymphocytic leukemia (CLL) is a heterogeneous B
cell malignancy characterized by the accumulation of
functionally incompetent B lymphocytes. Despite the
availability of highly effective treatments, CLL remains
incurable, and the factors contributing to disease
progression are not fully understood. Autoimmune
complications frequently arise in CLL patients and are
associated with poor clinical prognosis. This study
investigates the connection between plasma cell-mediated
autoimmunity and CLL progression using a mouse model that
expresses an active Receptor Activator of NF-κB (RANK) in B
cells (RK mice), where autoimmune manifestations coexist
with CLL. Transcriptional profiling of RANK-driven leukemic
cells revealed a more indolent form of CLL compared to the
classical TCL1 model. The discovery of near-identical CDR3
regions in both plasma and CLL cells of RK mice suggests a
shared progenitor and antigen driving both conditions.
Deletion of Blimp-1, which prevents plasma cell
differentiation, initially enhanced B1/CLL formation in
young mice but nearly halted CLL progression, highlighting
the significant influence of autoimmune complications on
disease outcomes. This research underscores the intertwined
nature of autoimmunity and CLL, suggesting that targeting
inflammatory pathways could offer therapeutic potential for
managing both conditions.},
keywords = {Animals / Leukemia, Lymphocytic, Chronic, B-Cell:
immunology / Leukemia, Lymphocytic, Chronic, B-Cell:
pathology / Autoimmunity / Mice / Disease Progression /
Disease Models, Animal / Humans / B-Lymphocytes: immunology
/ B-Lymphocytes: metabolism / Plasma Cells: immunology /
Plasma Cells: metabolism / Plasma Cells: pathology},
cin = {MU01},
ddc = {600},
cid = {I:(DE-He78)MU01-20160331},
pnm = {899 - ohne Topic (POF4-899)},
pid = {G:(DE-HGF)POF4-899},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:39900937},
pmc = {pmc:PMC11791097},
doi = {10.1038/s41598-025-86876-1},
url = {https://inrepo02.dkfz.de/record/298559},
}