Non-Hodgkin lymphoma and pre-existing conditions: spectrum, clinical characteristics and outcome in 213 children and adolescents
Andishe Attarbaschi,
Elisa Carraro,
Oussama Abla,
Shlomit Barzilai-Birenboim,
Simon Bomken,
Laurence Brugieres,
Eva Bubanska,
Birgit Burkhardt,
Alan K.S. Chiang,
Monika Csoka,
Alina Fedorova,
Janez Jazbec,
Edita Kabickova,
Zdenka Krenova,
Jelena Lazic,
Jan Loeffen,
Georg Mann,
Felix Niggli,
Natalia Miakova,
Tomoo Osumi,
Leila Ronceray,
Anne Uyttebroeck,
Denise Williams,
Wilhelm Woessmann,
Grazyna Wrobel,
Marta Pillon
Affiliations
Andishe Attarbaschi
Pediatric Hematology and Oncology, St. Anna Children’s Hospital, Medical University of Vienna, Austria
Elisa Carraro
Pediatric Hematology and Oncology, University of Padova, Italy
Oussama Abla
Depar tment of Pediatrics, Division of Hematology and Oncology, Hospital for Sick, Children, Toronto, Canada
Shlomit Barzilai-Birenboim
Pediatric Hematology and Oncology, Schneider Children’s, Medical Center of Israel, Petah-Tivka, Israel and Sackler Faculty of Medicine, Tel Aviv, University, Israel
Simon Bomken
Northern Institute for Cancer Research, Newcastle University, UK
Laurence Brugieres
Department of Pediatric Oncology, Institute Gustave-Roussy, Villejuif, France
Eva Bubanska
Department of Pediatric Oncology and Hematology, University Children’s Hospital, Banska Bystrica, Slovakia
Birgit Burkhardt
Pediatric Hematology and Oncology, University of Munster, Germany
Alan K.S. Chiang
Department of Pediatrics and Adolescent Medicine, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Queen Mary Hospital, Pokfulam, Hong Kong
Monika Csoka
Pediatric Hematology and Oncology, Semmelweis University, Budapest, Hungary
Alina Fedorova
Belarusian Research Center for Pediatric Oncology, Hematology and Immunology, Minsk, Belarus
Janez Jazbec
Division of Pediatrics, Hematology and Oncology, University Medical Center Ljubljana, Slovenia
Edita Kabickova
Pediatric Hematology and Oncology, Charles University and University Hospital Motol, Prague, Czech Republic
Zdenka Krenova
Pediatric Hematology and Oncology, University Hospital, Brno, Czech Republic
Jelena Lazic
Pediatric Hematology and Oncology, University Children’s Hospital, School of Medicine University of Belgrade, Serbia
Jan Loeffen
Pediatric Hematology and Oncology, Erasmus MC - Sophia Children’s Hospital, Rotterdam, the Netherlands
Georg Mann
Pediatric Hematology and Oncology, St. Anna Children’s Hospital, Medical University of Vienna, Austria
Felix Niggli
Pediatric Hematology and Oncology, University Hospital, Zurich, Switzerland
Natalia Miakova
Pediatric Hematology and Oncology, Federal Center for Pediatric Hematology, Oncology and Immunology, Moscow, Russia
Tomoo Osumi
Children’s Cancer Center, National Center for Child Health and Development, Tokyo, Japan
Leila Ronceray
Pediatric Hematology and Oncology, St. Anna Children’s Hospital, Medical University of Vienna, Austria
Anne Uyttebroeck
Pediatric Hematology and Oncology, University Hospitals Leuven, Belgium
Denise Williams
Pediatric Hematology and Oncology, Cambridge University Hospitals Foundation Trust, Addenbrooke’s Hospital, Cambridge, UK
Wilhelm Woessmann
Pediatric Hematology and Oncology, Justus Liebig University, Giessen, Germany
Grazyna Wrobel
Bone Marrow Transplantation and Pediatric Hematology and Oncology, Wroclaw Medical University, Poland
Marta Pillon
Pediatric Hematology and Oncology, University of Padova, Italy
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.
