% IMPORTANT: The following is UTF-8 encoded. This means that in the presence
% of non-ASCII characters, it will not work with BibTeX 0.99 or older.
% Instead, you should use an up-to-date BibTeX implementation like “bibtex8” or
% “biber”.
@ARTICLE{Kempter:299835,
author = {T. Kempter and P. Richter-Pechańska and K. Michel and T.
Rausch and B. Erarslan-Uysal and C. Eckert and M. Zimmermann
and M. Stanulla and M. Schrappe and G. Cario and S. Köhrer
and A. Attarbaschi and J. O. Korbel and J. B. Kunz and A.
Kulozik$^*$},
title = {{S}ubclonal {TP}53 and {KRAS} variants combined with poor
treatment response identify ultrahigh-risk pediatric
patients with {T}-{ALL}.},
journal = {Blood advances},
volume = {9},
number = {6},
issn = {2473-9529},
address = {Washington, DC},
publisher = {American Society of Hematology},
reportid = {DKFZ-2025-00572},
pages = {1267 - 1279},
year = {2025},
note = {#LA:A400#},
abstract = {Variations in the TP53 and KRAS genes indicate a
particularly adverse prognosis in relapsed pediatric T-cell
acute lymphoblastic leukemia (T-ALL). We hypothesized that
these variations might be subclonally present at disease
onset and contribute to relapse risk. To test this, we
examined 2 cohorts of children diagnosed with T-ALL: cohort
1 with 81 patients who relapsed and 79 who matched
nonrelapsing controls, and cohort 2 with 226 consecutive
patients, 30 of whom relapsed. In cohort 1, targeted
sequencing revealed TP53 clonal and subclonal variants in 6
of 81 relapsing patients but none in the nonrelapsing group
(P = .014). KRAS alterations were found in 9 of 81 relapsing
patients compared with 2 of 79 nonrelapsing patients (P =
.032). Survival analysis showed that none of the relapsed
patients with TP53 and/or KRAS alterations survived, whereas
19 of 67 relapsed patients without such variants did, with a
minimum follow-up time of 3 years (P = .023). In cohort 2,
none of the relapsing patients but 10 of 196 nonrelapsing
patients carried TP53 or KRAS variants, indicating that
mutation status alone does not predict poor prognosis. All
10 nonrelapsing patients with mutations had a favorable
early treatment response. Among the total cohort of 386
patients, 188 showed poor treatment response, of whom 69
relapsed. Of these poor responders, 9 harbored TP53 or KRAS
variants. In conclusion, subclonal TP53 and KRAS alterations
identified at the time of initial diagnosis, along with a
poor treatment response, characterize a subset of children
with T-ALL who face a dismal prognosis and who may benefit
from alternative treatment approaches.},
keywords = {Humans / Tumor Suppressor Protein p53: genetics /
Proto-Oncogene Proteins p21(ras): genetics / Child /
Precursor T-Cell Lymphoblastic Leukemia-Lymphoma: genetics /
Precursor T-Cell Lymphoblastic Leukemia-Lymphoma: mortality
/ Precursor T-Cell Lymphoblastic Leukemia-Lymphoma:
diagnosis / Precursor T-Cell Lymphoblastic
Leukemia-Lymphoma: therapy / Male / Female / Child,
Preschool / Prognosis / Adolescent / Infant / Mutation /
Treatment Outcome / Tumor Suppressor Protein p53 (NLM
Chemicals) / Proto-Oncogene Proteins p21(ras) (NLM
Chemicals) / KRAS protein, human (NLM Chemicals) / TP53
protein, human (NLM Chemicals)},
cin = {A400},
ddc = {610},
cid = {I:(DE-He78)A400-20160331},
pnm = {311 - Zellbiologie und Tumorbiologie (POF4-311)},
pid = {G:(DE-HGF)POF4-311},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:39808796},
doi = {10.1182/bloodadvances.2024014209},
url = {https://inrepo02.dkfz.de/record/299835},
}