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Hematopoietic Stem Cell Transplantation Positively Affects the Natural History of Cancer in Nijmegen Breakage Syndrome
B. Wolska-Kusnierz, A. Pastorczak, W. Fendler, A. Wakulinska, B. Dembowska-Baginska, E. Heropolitanska-Pliszka, B. Piątosa, B. Pietrucha, K. Kałwak, M. Ussowicz, A. Pieczonka, K. Drabko, M. Lejman, S. Koltan, J. Gozdzik, J. Styczynski, A....
Language English Country United States
Document type Journal Article, Multicenter Study, Research Support, Non-U.S. Gov't
NLK
Free Medical Journals
from 1995 to 1 year ago
Freely Accessible Science Journals
from 1995
Open Access Digital Library
from 1995-01-01
Open Access Digital Library
from 1995-01-01
- MeSH
- Child MeSH
- Adult MeSH
- Incidence MeSH
- Kaplan-Meier Estimate MeSH
- Cohort Studies MeSH
- Comorbidity MeSH
- Humans MeSH
- Adolescent MeSH
- Young Adult MeSH
- Neoplasms epidemiology therapy MeSH
- Follow-Up Studies MeSH
- Prevalence MeSH
- Nijmegen Breakage Syndrome epidemiology MeSH
- Hematopoietic Stem Cell Transplantation methods MeSH
- Check Tag
- Child MeSH
- Adult MeSH
- Humans MeSH
- Adolescent MeSH
- Young Adult MeSH
- Publication type
- Journal Article MeSH
- Multicenter Study MeSH
- Research Support, Non-U.S. Gov't MeSH
- Geographicals
- Poland MeSH
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.
Belarusian Research Center for Pediatric Oncology and Hematology Minsk Belarus
Department of Biostatistics and Translational Medicine Medical University of Lodz Lodz Poland
Department of Hematology Oncology and Internal Medicine Medical University of Warsaw Warsaw Poland
Department of Immunology Children's Memorial Health Institute Warsaw Poland
Department of Medical Genetics The Children's Memorial Health Institute Warsaw Poland
Department of Oncology Children's Memorial Health Institute Warsaw Poland
Department of Pediatric Hematology and Oncology Oslo University Hospital Oslo Norway
Department of Pediatric Hematology and Oncology University Hospital Heidelberg Germany
Department of Pediatric Hematology Oncology and Transplantology Medical University of Lublin Poland
Department of Pediatric Oncology Poznan University of Medical Sciences Poznan Poland
Department of Pediatrics Hannover Medical School Hannover Germany
Department of Radiation Oncology Dana Farber Cancer Institute Boston Massachusetts
Department Pediatrics Oncology and Hematology Medical University of Lodz Lodz Poland
Dr von Hauner University Children's Hospital Ludwig Maximilians University Munich Germany
Great Ormond Street Hospital for Children NHS Foundation Trust London United Kingdom
Histocompatibility Laboratory Children's Memorial Health Institute Warsaw Poland
Pediatric Department St Olav University Hospital Trondheim Norway
References provided by Crossref.org
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