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-Münster 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.

Belarusian Research Center for Pediatric Oncology Hematology and Immunology Minsk Belarus

Bone Marrow Transplantation and Pediatric Hematology and Oncology Wroclaw Medical University Poland

Children's Cancer Center National Center for Child Health and Development Tokyo Japan

Depar tment of Pediatrics Division of Hematology and Oncology Hospital for Sick Children Toronto Canada

Department of Pediatric Oncology and Hematology University Children's Hospital Banska Bystrica Slovakia

Department of Pediatric Oncology Institute Gustave Roussy Villejuif France

Department of Pediatrics and Adolescent Medicine Li Ka Shing Faculty of Medicine The University of Hong Kong Queen Mary Hospital Pokfulam Hong Kong

Division of Pediatrics Hematology and Oncology University Medical Center Ljubljana Slovenia

Northern Institute for Cancer Research Newcastle University UK

Pediatric Hematology and Oncology Cambridge University Hospitals Foundation Trust Addenbrooke's Hospital Cambridge UK

Pediatric Hematology and Oncology Charles University and University Hospital Motol Prague Czech Republic

Pediatric Hematology and Oncology Erasmus MC Sophia Children's Hospital Rotterdam the Netherlands

Pediatric Hematology and Oncology Federal Center for Pediatric Hematology Oncology and Immunology Moscow Russia

Pediatric Hematology and Oncology Justus Liebig University Giessen Germany

Pediatric Hematology and Oncology Schneider Children's Medical Center of Israel Petah Tivka Israel and Sackler Faculty of Medicine Tel Aviv University Israel

Pediatric Hematology and Oncology Semmelweis University Budapest Hungary

Pediatric Hematology and Oncology St Anna Children's Hospital Medical University of Vienna Austria

Pediatric Hematology and Oncology University Children's Hospital School of Medicine University of Belgrade Serbia

Pediatric Hematology and Oncology University Hospital Brno Czech Republic

Pediatric Hematology and Oncology University Hospital Zurich Switzerland

Pediatric Hematology and Oncology University Hospitals Leuven Belgium

Pediatric Hematology and Oncology University of Munster Germany

Pediatric Hematology and Oncology University of Padova Italy

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