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@ARTICLE{RichterPechaska:179973,
author = {P. Richter-Pechańska and J. B. Kunz and T. Rausch and B.
Erarslan-Uysal and B. Bornhauser and V. Frismantas and Y.
Assenov$^*$ and M. Zimmermann and M. Happich and C. von
Knebel-Doeberitz and N. von Neuhoff and R. Köhler and M.
Stanulla and M. Schrappe and G. Cario and G. Escherich and
R. Kirschner-Schwabe$^*$ and C. Eckert$^*$ and S. Avigad and
S. M. Pfister$^*$ and M. U. Muckenthaler and J.-P. Bourquin
and J. O. Korbel and A. Kulozik$^*$},
title = {{P}ediatric {T}-{ALL} type-1 and type-2 relapses develop
along distinct pathways of clonal evolution.},
journal = {Leukemia},
volume = {36},
number = {7},
issn = {0887-6924},
address = {London},
publisher = {Springer Nature},
reportid = {DKFZ-2022-01013},
pages = {1759-1768},
year = {2022},
note = {#LA:B062# / 2022 Jul;36(7):1759-1768},
abstract = {The mechanisms underlying T-ALL relapse remain essentially
unknown. Multilevel-omics in 38 matched pairs of initial and
relapsed T-ALL revealed 18 $(47\%)$ type-1 (defined by being
derived from the major ancestral clone) and 20 $(53\%)$
type-2 relapses (derived from a minor ancestral clone). In
both types of relapse, we observed known and novel drivers
of multidrug resistance including MDR1 and MVP, NT5C2 and
JAK-STAT activators. Patients with type-1 relapses were
specifically characterized by IL7R upregulation. In
remarkable contrast, type-2 relapses demonstrated (1)
enrichment of constitutional cancer predisposition gene
mutations, (2) divergent genetic and epigenetic remodeling,
and (3) enrichment of somatic hypermutator phenotypes,
related to BLM, BUB1B/PMS2 and TP53 mutations. T-ALLs that
later progressed to type-2 relapses exhibited a complex
subclonal architecture, unexpectedly, already at the time of
initial diagnosis. Deconvolution analysis of ATAC-Seq
profiles showed that T-ALLs later developing into type-1
relapses resembled a predominant immature thymic T-cell
population, whereas T-ALLs developing into type-2 relapses
resembled a mixture of normal T-cell precursors. In sum, our
analyses revealed fundamentally different mechanisms driving
either type-1 or type-2 T-ALL relapse and indicate that
differential capacities of disease evolution are already
inherent to the molecular setup of the initial leukemia.},
cin = {B062 / HD01 / BE01},
ddc = {610},
cid = {I:(DE-He78)B062-20160331 / I:(DE-He78)HD01-20160331 /
I:(DE-He78)BE01-20160331},
pnm = {312 - Funktionelle und strukturelle Genomforschung
(POF4-312)},
pid = {G:(DE-HGF)POF4-312},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:35585141},
doi = {10.1038/s41375-022-01587-0},
url = {https://inrepo02.dkfz.de/record/179973},
}