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@ARTICLE{Jahn:166582,
author = {N. Jahn and T. Terzer$^*$ and E. Sträng and A. Dolnik and
S. Cocciardi and E. Panina and A. Corbacioglu and J. Herzig
and D. Weber and A. Schrade and K. Götze and T. Schröder
and M. Lübbert and D. Wellnitz and E. Koller and R. F.
Schlenk and V. I. Gaidzik and P. Paschka and F. G. Rücker
and M. Heuser and F. Thol and A. Ganser and A. Benner$^*$
and H. Döhner and L. Bullinger and K. Döhner},
title = {{G}enomic heterogeneity in core-binding factor acute
myeloid leukemia and its clinical implication.},
journal = {Blood advances},
volume = {4},
number = {24},
issn = {2473-9537},
address = {Washington, DC},
publisher = {American Society of Hematology},
reportid = {DKFZ-2020-03025},
pages = {6342 - 6352},
year = {2020},
abstract = {Core-binding factor (CBF) acute myeloid leukemia (AML)
encompasses AML with inv(16)(p13.1q22) and AML with
t(8;21)(q22;q22.1). Despite sharing a common pathogenic
mechanism involving rearrangements of the CBF
transcriptional complex, there is growing evidence for
considerable genotypic heterogeneity. We comprehensively
characterized the mutational landscape of 350 adult CBF-AML
[inv(16): n = 160, t(8;21): n = 190] performing targeted
sequencing of 230 myeloid cancer-associated genes. Apart
from common mutations in signaling genes, mainly NRAS, KIT,
and FLT3, both CBF-AML entities demonstrated a remarkably
diverse pattern with respect to the underlying cooperating
molecular events, in particular in genes encoding for
epigenetic modifiers and the cohesin complex. In addition,
recurrent mutations in novel collaborating candidate genes
such as SRCAP $(5\%$ overall) and DNM2 $(6\%$ of t(8;21)
AML) were identified. Moreover, aberrations altering
transcription and differentiation occurred at earlier
leukemic stages and preceded mutations impairing
proliferation. Lasso-penalized models revealed an inferior
prognosis for t(8;21) AML, trisomy 8, as well as FLT3 and
KIT exon 17 mutations, whereas NRAS and WT1 mutations
conferred superior prognosis. Interestingly, clonal
heterogeneity was associated with a favorable prognosis.
When entering mutations by functional groups in the model,
mutations in genes of the methylation group (ie, DNMT3A,
TET2) had a strong negative prognostic impact.},
cin = {C060},
ddc = {610},
cid = {I:(DE-He78)C060-20160331},
pnm = {313 - Cancer risk factors and prevention (POF3-313)},
pid = {G:(DE-HGF)POF3-313},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:33351131},
doi = {10.1182/bloodadvances.2020002673},
url = {https://inrepo02.dkfz.de/record/166582},
}