| WOLSKA-KUSNIERZ, Beata, Agata PASTORCZAK, Wojciech FENDLER, Anna WAKULINSKA, Bozena DEMBOWSKA-BAGINSKA, Edyta HEROPOLITANSKA-PLISZKA, Barbara PIATOSA, Barbara PIETRUCHA, Krzysztof KALWAK, Marek USSOWICZ, Anna PIECZONKA, Katarzyna DRABKO, Monika LEJMAN, Sylwia KOLTAN, Jolanta GOZDZIK, Jan STYCZYNSKI, Alina FEDOROVA, Natalia MIAKOVA, Elena DERIPAPA, Larysa KOSTYUCHENKO, Zdenka KŘENOVÁ, Eva HLAVÁČKOVÁ, Andrew GENNERY, Karl-Walter SYKORA, Sujal GHOSH, Michael H ALBERT, Dmitry BALASHOV, Mary EAPEN, Peter SVEC, Markus G SEIDEL, Sara Sebnem KILIC, Agnieszka TOMASZEWSKA, Ewa WIESIK-SZEWCZYK, Alexandra KREINS, Johann GREIL, Jochen BUECHNER, Bendik LUND, Hanna GREGOREK, Krystyna CHRZANOWSKA and Wojciech MLYNARSKI. Hematopoietic stem cell transplantation positively affects the natural history of cancer in Nijmegen breakage syndrome. Clinical Cancer Research. Philadelphia: American Association for Cancer Research, 2021, vol. 27, No 2, p. 575-584. ISSN 1078-0432. Available from: https://dx.doi.org/10.1158/1078-0432.CCR-20-2574. |
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@article{1697377,
author = {WolskaandKusnierz, Beata and Pastorczak, Agata and Fendler, Wojciech and Wakulinska, Anna and DembowskaandBaginska, Bozena and HeropolitanskaandPliszka, Edyta and Piatosa, Barbara and Pietrucha, Barbara and Kalwak, Krzysztof and Ussowicz, Marek and Pieczonka, Anna and Drabko, Katarzyna and Lejman, Monika and Koltan, Sylwia and Gozdzik, Jolanta and Styczynski, Jan and Fedorova, Alina and Miakova, Natalia and Deripapa, Elena and Kostyuchenko, Larysa and Křenová, Zdenka and Hlaváčková, Eva and Gennery, Andrew and Sykora, KarlandWalter and Ghosh, Sujal and Albert, Michael H and Balashov, Dmitry and Eapen, Mary and Svec, Peter and Seidel, Markus G and Kilic, Sara Sebnem and Tomaszewska, Agnieszka and WiesikandSzewczyk, Ewa and Kreins, Alexandra and Greil, Johann and Buechner, Jochen and Lund, Bendik and Gregorek, Hanna and Chrzanowska, Krystyna and Mlynarski, Wojciech},
article_location = {Philadelphia},
article_number = {2},
doi = {http://dx.doi.org/10.1158/1078-0432.CCR-20-2574},
keywords = {Nijmegen breakage syndrome (NBS)},
language = {eng},
issn = {1078-0432},
journal = {Clinical Cancer Research},
title = {Hematopoietic stem cell transplantation positively affects the natural history of cancer in Nijmegen breakage syndrome},
url = {https://clincancerres.aacrjournals.org/content/early/2020/10/20/1078-0432.CCR-20-2574.abstract},
volume = {27},
year = {2021}
}
TY - JOUR
ID - 1697377
AU - Wolska-Kusnierz, Beata - Pastorczak, Agata - Fendler, Wojciech - Wakulinska, Anna - Dembowska-Baginska, Bozena - Heropolitanska-Pliszka, Edyta - Piatosa, Barbara - Pietrucha, Barbara - Kalwak, Krzysztof - Ussowicz, Marek - Pieczonka, Anna - Drabko, Katarzyna - Lejman, Monika - Koltan, Sylwia - Gozdzik, Jolanta - Styczynski, Jan - Fedorova, Alina - Miakova, Natalia - Deripapa, Elena - Kostyuchenko, Larysa - Křenová, Zdenka - Hlaváčková, Eva - Gennery, Andrew - Sykora, Karl-Walter - Ghosh, Sujal - Albert, Michael H - Balashov, Dmitry - Eapen, Mary - Svec, Peter - Seidel, Markus G - Kilic, Sara Sebnem - Tomaszewska, Agnieszka - Wiesik-Szewczyk, Ewa - Kreins, Alexandra - Greil, Johann - Buechner, Jochen - Lund, Bendik - Gregorek, Hanna - Chrzanowska, Krystyna - Mlynarski, Wojciech
PY - 2021
TI - Hematopoietic stem cell transplantation positively affects the natural history of cancer in Nijmegen breakage syndrome
JF - Clinical Cancer Research
VL - 27
IS - 2
SP - 575-584
EP - 575-584
PB - American Association for Cancer Research
SN - 10780432
KW - Nijmegen breakage syndrome (NBS)
UR - https://clincancerres.aacrjournals.org/content/early/2020/10/20/1078-0432.CCR-20-2574.abstract
L2 - https://clincancerres.aacrjournals.org/content/early/2020/10/20/1078-0432.CCR-20-2574.abstract
N2 - Among 241 patients with NBS enrolled in the study from 11 countries, 151 (63.0%) patients were diagnosed with cancer. Incidence rates for primary and secondary cancer, tumor characteristics, and risk factors affecting overall survival (OS) were estimated. The cumulative cancer incidence was 40.21% ± 3.5% and 77.78% ± 3.4% at 10 years and 20 years of follow-up, respectively. Most of the tumors n = 95 (62.9%) were non-Hodgkin lymphomas. Overall, 20 (13.2%) secondary malignancies occurred at a median age of 18 (interquartile range, 13.7–21.5) years. The probability of 20 year overall survival (OS) for the whole cohort was 44.6% ± 4.5%. Patients who developed cancer had a shorter 20 year OS than those without malignancy (29.6% vs. 86.2%; P < ). A total of 49 patients with NBS underwent HSCT, including 14 patients transplanted before malignancy. Patients with NBS with diagnosed cancer who received HSCT had higher 20 year OS than those who did not (42.7% vs. 30.3%; P = 0.038, respectively). In the group of patients who underwent preemptive transplantation, only 1 patient developed cancer, which is 6.7 times lower as compared with nontransplanted patients [incidence rate ratio 0.149 (95% confidence interval, 0.138–0.162); P < 0.0001].
ER -
WOLSKA-KUSNIERZ, Beata, Agata PASTORCZAK, Wojciech FENDLER, Anna WAKULINSKA, Bozena DEMBOWSKA-BAGINSKA, Edyta HEROPOLITANSKA-PLISZKA, Barbara PIATOSA, Barbara PIETRUCHA, Krzysztof KALWAK, Marek USSOWICZ, Anna PIECZONKA, Katarzyna DRABKO, Monika LEJMAN, Sylwia KOLTAN, Jolanta GOZDZIK, Jan STYCZYNSKI, Alina FEDOROVA, Natalia MIAKOVA, Elena DERIPAPA, Larysa KOSTYUCHENKO, Zdenka KŘENOVÁ, Eva HLAVÁČKOVÁ, Andrew GENNERY, Karl-Walter SYKORA, Sujal GHOSH, Michael H ALBERT, Dmitry BALASHOV, Mary EAPEN, Peter SVEC, Markus G SEIDEL, Sara Sebnem KILIC, Agnieszka TOMASZEWSKA, Ewa WIESIK-SZEWCZYK, Alexandra KREINS, Johann GREIL, Jochen BUECHNER, Bendik LUND, Hanna GREGOREK, Krystyna CHRZANOWSKA and Wojciech MLYNARSKI. Hematopoietic stem cell transplantation positively affects the natural history of cancer in Nijmegen breakage syndrome. \textit{Clinical Cancer Research}. Philadelphia: American Association for Cancer Research, 2021, vol.~27, No~2, p.~575-584. ISSN~1078-0432. Available from: https://dx.doi.org/10.1158/1078-0432.CCR-20-2574.
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