Browsing by Author "Pastorczak, Agata"

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    Hematopoietic stem cell transplantation positively affects the natural history of cancer in nijmegen breakage syndrome
    (Amer Assoc Cancer Research, 2021-01-15) 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; Krenova, Zdenka; Hlavackova, Eva; Gennery, Andrew R.; Sykora, Karl-Walter; Ghosh, Sujal; Albert, Michael H.; Balashov, Dmitry; Eapen, Mary; Svec, Peter; Seidel, Markus G.; Tomaszewska, Agnieszka; Wiesik-Szewczyk, Ewa; Kreins, Alexandra; Greil, Johann; Buechner, Jochen; Lund, Bendik; Gregorek, Hanna; Chrzanowska, Krystyna; Mlynarski, Wojciech; Kilic, Sara S.; KILIÇ GÜLTEKİN, SARA ŞEBNEM; Bursa Uludağ Üniversitesi/Çocuk Sağlığı ve Hastalıkları Anabilim Dalı.; 0000-0003-4863-4443; 0000-0003-3089-6947; 0000-0002-5083-9168; 0000-0002-2561-0636; 0000-0001-5519-2730; 0000-0001-8949-047X; 0000-0002-9723-8351; 0000-0003-1174-5799; 0000-0001-5725-4835; 0000-0001-5922-4242; 0000-0002-7094-9129; 0000-0002-8760-0775; 0000-0002-3158-119X; 0000-0002-0262-1359; 0000-0001-9143-3263; 0000-0001-6590-5802; 0000-0001-6193-4243; 0000-0002-7647-2253; 0000-0003-0981-8661; 0000-0001-8571-2581; 0000-0001-8509-4453; 0000-0001-8748-5837; 0000-0001-5848-4501; 0000-0003-2714-5851; S-9959-2016; T-7487-2019; B-4557-2018; P-1827-2019; AAH-1658-2021; N-9951-2017; AAD-5720-2020; S-9592-2016
    Purpose: Nijmegen breakage syndrome (NBS) is a DNA repair disorder with a high predisposition to hematologic malignancies.Experimental Design: We describe the natural history of NBS, including cancer incidence, risk of death, and the potential effectiveness of hematopoietic stem cell transplantation (HSCT) in preventing both pathologies: malignancy and immunodeficiency.Results: 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 < 10(-5)). 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].Conclusions: There is a beneficial effect of HSCT on the long-term survival of patients with NBS transplanted in their first complete remission of cancer.