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@ARTICLE{Cocciardi:297977,
author = {S. Cocciardi and M. Saadati$^*$ and N. Weiß and D. Späth
and S. Kapp-Schwoerer and I. Schneider and A. Meid and V. I.
Gaidzik and S. Skambraks and W. Fiedler and M. W. M. Kühn
and U. Germing and K. T. Mayer and M. Lübbert and E.
Papaemmanuil and F. Thol and M. Heuser and A. Ganser and L.
Bullinger and A. Benner$^*$ and H. Döhner and K. Döhner},
title = {{I}mpact of myelodysplasia-related and additional gene
mutations in intensively treated patients with
{NPM}1-mutated {AML}.},
journal = {HemaSphere},
volume = {9},
number = {1},
issn = {2572-9241},
address = {Hoboken},
publisher = {John Wiley $\&$ Sons Ltd.},
reportid = {DKFZ-2025-00152},
pages = {e70060},
year = {2025},
abstract = {This study aimed to evaluate the impact of the
myelodysplasia-related gene (MRG) as well as additional gene
mutations on outcomes in intensively treated patients with
NPM1-mutated (NPM1 mut) AML. Targeted DNA sequencing of 263
genes was performed in 568 NPM1 mut AML patients (median
age: 59 years) entered into the prospective AMLSG 09-09
treatment trial. Most commonly co-mutated genes were DNMT3A
$(49.8\%),$ FLT3-TKD $(25.9\%),$ PTPN11 $(24.8\%),$ NRAS
$(22.7\%),$ TET2 $(21.7\%),$ IDH2 $(21.3\%),$ IDH1 $(18\%),$
and FLT3-ITD $(17.3\%).$ MRG mutations were identified in
$18.1\%$ of cases (18-60 years: $9.8\%;$ >60 years:
$28.7\%).$ When focusing on the 470 patients with 2022 ELN
favorable-risk NPM1 mut AML, multivariable analysis for
event-free survival (EFS) identified age (p < 0.001), DNMT3A
R882 (p < 0.001), IDH1 (p = 0.007), and MRG mutations (p =
0.03) as unfavorable factors, cohesin gene co-mutations (p =
0.001) and treatment with gemtuzumab ozogamicin (p = 0.007)
as favorable factors. Restricting the analysis to a subset
of CR/CRi patients with available data on NPM1 mut
measurable residual disease (MRD) status in blood post cycle
2 in the model, MRG mutations lost their significant effect,
whereas DNMT3A R882, MYC, and cohesin gene mutations
retained the adverse and favorable effects. For OS, age (p <
0.001), DNMT3A R882 (p = 0.042), IDH1 (p = 0.045), and KRAS
(0.003) mutations were unfavorable factors, sole favorable
factor was IDH2 co-mutation (p = 0.037). In 2022 ELN
favorable-risk NPM1 mut AML, MRG mutations are associated
with inferior EFS; however, this effect is no longer present
when considering NPM1 mut MRD status post cycle 2; DNMT3A
R882 and MYC mutations remained adverse, and cohesin gene
mutations favorable prognostic factors independent of the
NPM1 mut MRD status.},
cin = {C060},
ddc = {610},
cid = {I:(DE-He78)C060-20160331},
pnm = {313 - Krebsrisikofaktoren und Prävention (POF4-313)},
pid = {G:(DE-HGF)POF4-313},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:39816531},
pmc = {pmc:PMC11733593},
doi = {10.1002/hem3.70060},
url = {https://inrepo02.dkfz.de/record/297977},
}