期刊
HAEMATOLOGICA
卷 101, 期 12, 页码 1581-1591出版社
FERRATA STORTI FOUNDATION
DOI: 10.3324/haematol.2016.147116
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资金
- Cancer Research United Kingdom
- Forschungshilfe Station Peiper (BFM Germany)
- St. Anna Kinderkrebsforschung (BFM Austria)
- Czech Ministry of Health [00064203, 65269705]
- National Program of Sustainability II (Czech Republic) [LQ1605]
- Ministry of Health, Labour and Welfare of Japan (JPLSG)
- National Institute for Health Research [CL-2012-01-002] Funding Source: researchfish
Children and adolescents with pre-existing conditions such as DNA repair defects or other primary immunodeficiencies have an increased risk of non-Hodgkin lymphoma. However, large-scale data on patients with non-Hodgkin lymphoma and their entire spectrum of pre-existing conditions are scarce. A retrospective multinational study was conducted by means of questionnaires sent out to the national study groups or centers, by the two largest consortia in childhood non-Hodgkin lymphoma, the European Intergroup for Childhood non-Hodgkin Lymphoma, and the international Berlin-Frankfurt-Munster Study Group. The study identified 213 patients with non-Hodgkin lymphoma and a pre-existing condition. Four subcategories were established: a) cancer predisposition syndromes (n=124, 58%); b) primary immunodeficiencies not further specified (n=27, 13%); c) genetic diseases with no increased cancer risk (n=40, 19%); and d) non-classifiable conditions (n=22, 10%). Seventy-nine of 124 (64%) cancer predispositions were reported in groups with more than 20 patients: ataxia telangiectasia (n=32), Nijmegen breakage syndrome (n=26), constitutional mismatch repair deficiency (n=21). For the 151 patients with a known cancer risk, 5-year event-free survival and overall survival rates were 40%+/- 4% and 51%+/- 4%, respectively. Five-year cumulative incidences of progression/relapse and treatment-related death as a first event were 22%+/- 4% and 24%+/- 4%, respectively. Ten-year incidence of second malignancy was 24%+/- 5% and 7-year overall survival of the 21 patients with a second malignancy was 41%+/- 11%. Patients with non-Hodgkin lymphoma and pre-existing conditions have an inferior survival rate with a large proportion of therapy-related deaths compared to patients with non-Hodgkin lymphoma and no pre-existing conditions. They may require special vigilance when receiving standard or modified/reduced-intensity chemotherapy or when undergoing allogeneic stem cell transplantation.
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