J 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)

Štítky

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].