2021
Hematopoietic stem cell transplantation positively affects the natural history of cancer in Nijmegen breakage syndrome
WOLSKA-KUSNIERZ, Beata, Agata PASTORCZAK, Wojciech FENDLER, Anna WAKULINSKA, Bozena DEMBOWSKA-BAGINSKA et. al.Základní údaje
Originální název
Hematopoietic stem cell transplantation positively affects the natural history of cancer in Nijmegen breakage syndrome
Autoři
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 (203 Česká republika), Eva HLAVÁČKOVÁ (203 Česká republika, domácí), 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 a Wojciech MLYNARSKI (garant)
Vydání
Clinical Cancer Research, Philadelphia, American Association for Cancer Research, 2021, 1078-0432
Další údaje
Jazyk
angličtina
Typ výsledku
Článek v odborném periodiku
Obor
30204 Oncology
Stát vydavatele
Spojené státy
Utajení
není předmětem státního či obchodního tajemství
Odkazy
Impakt faktor
Impact factor: 13.801
Kód RIV
RIV/00216224:14110/21:00120821
Organizační jednotka
Lékařská fakulta
UT WoS
000617323400025
Klíčová slova anglicky
Nijmegen breakage syndrome (NBS)
Příznaky
Mezinárodní význam, Recenzováno
Změněno: 8. 4. 2021 08:02, Mgr. Tereza Miškechová
Anotace
V originále
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].