title: Non-Hodgkin lymphoma and pre-existing conditions: spectrum, clinical characteristics and outcome in 213 children and adolescents.
abstract: 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.
authors: Pediatric Hematology and Oncology, St. Anna Children's Hospital, Medical University of Vienna, Austria andishe.attarbaschi@stanna.at.
authors: Andishe
authors: A
authors: Attarbaschi
authors: Pediatric Hematology and Oncology, University of Padova, Italy.
authors: Elisa
authors: E
authors: Carraro
authors: Depar tment of Pediatrics, Division of Hematology and Oncology, Hospital for Sick, Children, Toronto, Canada.
authors: Oussama
authors: O
authors: Abla
authors: Pediatric Hematology and Oncology, Schneider Children's, Medical Center of Israel, Petah-Tivka, Israel and Sackler Faculty of Medicine, Tel Aviv, University, Israel.
authors: Shlomit
authors: S
authors: Barzilai-Birenboim
authors: Northern Institute for Cancer Research, Newcastle University, UK.
authors: Simon
authors: S
authors: Bomken
authors: Department of Pediatric Oncology, Institute Gustave-Roussy, Villejuif, France.
authors: Laurence
authors: L
authors: Brugieres
authors: Department of Pediatric Oncology and Hematology, University Children's Hospital, Banska Bystrica, Slovakia.
authors: Eva
authors: E
authors: Bubanska
authors: Pediatric Hematology and Oncology, University of Munster, Germany.
authors: Birgit
authors: B
authors: Burkhardt
authors: Department of Pediatrics and Adolescent Medicine, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Queen Mary Hospital, Pokfulam, Hong Kong.
authors: Alan K S
authors: AK
authors: Chiang
authors: Pediatric Hematology and Oncology, Semmelweis University, Budapest, Hungary.
authors: Monika
authors: M
authors: Csoka
authors: Belarusian Research Center for Pediatric Oncology, Hematology and Immunology, Minsk, Belarus.
authors: Alina
authors: A
authors: Fedorova
authors: Division of Pediatrics, Hematology and Oncology, University Medical Center Ljubljana, Slovenia.
authors: Janez
authors: J
authors: Jazbec
authors: Pediatric Hematology and Oncology, Charles University and University Hospital Motol, Prague, Czech Republic.
authors: Edita
authors: E
authors: Kabickova
authors: Pediatric Hematology and Oncology, University Hospital, Brno, Czech Republic.
authors: Zdenka
authors: Z
authors: Krenova
authors: Pediatric Hematology and Oncology, University Children's Hospital, School of Medicine University of Belgrade, Serbia.
authors: Jelena
authors: J
authors: Lazic
authors: Pediatric Hematology and Oncology, Erasmus MC - Sophia Children's Hospital, Rotterdam, the Netherlands.
authors: Jan
authors: J
authors: Loeffen
authors: Pediatric Hematology and Oncology, St. Anna Children's Hospital, Medical University of Vienna, Austria.
authors: Georg
authors: G
authors: Mann
authors: Pediatric Hematology and Oncology, University Hospital, Zurich, Switzerland.
authors: Felix
authors: F
authors: Niggli
authors: Pediatric Hematology and Oncology, Federal Center for Pediatric Hematology, Oncology and Immunology, Moscow, Russia.
authors: Natalia
authors: N
authors: Miakova
authors: Children's Cancer Center, National Center for Child Health and Development, Tokyo, Japan.
authors: Tomoo
authors: T
authors: Osumi
authors: Pediatric Hematology and Oncology, St. Anna Children's Hospital, Medical University of Vienna, Austria.
authors: Leila
authors: L
authors: Ronceray
authors: Pediatric Hematology and Oncology, University Hospitals Leuven, Belgium.
authors: Anne
authors: A
authors: Uyttebroeck
authors: Pediatric Hematology and Oncology, Cambridge University Hospitals Foundation Trust, Addenbrooke's Hospital, Cambridge, UK.
authors: Denise
authors: D
authors: Williams
authors: Pediatric Hematology and Oncology, Justus Liebig University, Giessen, Germany.
authors: Wilhelm
authors: W
authors: Woessmann
authors: Bone Marrow Transplantation and Pediatric Hematology and Oncology, Wroclaw Medical University, Poland.
authors: Grazyna
authors: G
authors: Wrobel
authors: Pediatric Hematology and Oncology, University of Padova, Italy.
authors: Marta
authors: M
authors: Pillon
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D015897:Comorbidity; D018450:Disease Progression; D004198:Disease Susceptibility; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D008228:Lymphoma, Non-Hodgkin / Q000175:diagnosis / Q000453:epidemiology* / Q000401:mortality / Q000628:therapy; D008297:Male; D016609:Neoplasms, Second Primary / Q000453:epidemiology / Q000209:etiology; D062486:Public Health Surveillance; D012008:Recurrence; D016896:Treatment Outcome