title: Non-Hodgkin lymphoma in children with an associated inherited condition: A retrospective analysis of the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP).
abstract: BACKGROUND
  Inherited conditions affecting genetic aberration, viral oncogenesis, reduced immune surveillance, and long-lasting antigen stimulation may build the way to lymphomagenesis in humans.
  
  
  METHODS
  We extracted from the database of 4 consecutive trials for pediatric non-Hodgkin lymphoma (NHL) all cases with an associated genetic disease.
  
  
  RESULTS
  Among 1,430 patients, 34 (2.4%) had an associated inherited condition and a mature B-lineage (n = 28), anaplastic large cell lymphoma (n = 4), or T-lineage (n = 2) NHL. Their median age at the diagnosis was 9.3 years (range, 2.6-17.8 years). In 14 cases (41%) the underlying condition was considered to be a potential cause for undue toxicity if the expected therapy was applied. Thus, treatment modification had been planned in advance. The overall survival was 89% (standard error [SE] 1%), 73% (SE 10%), and 73% (SE 23%) at 3 years for registered patients with no inherited condition associated, with genetic abnormalities and with underlying condition causing an immune deficiency, respectively (P = 0.003).
  
  
  CONCLUSIONS
  In our cohort, patients with NHL with an underlying constitutional condition represent the 2.4% of the cases. In the subset of patients with primary immune deficiency, which may have contributed to lymphomagenesis, allogeneic hematopoietic stem cell transplantation may be required. In the remaining patients, the association with lymphoma remains apparently unexplained and could be not causative. Detailed reporting of such cases may contribute to disclose even rare and fully unexpected association, which may have implications for research in the field of lymphomagenesis.
authors.affiliation: Azienda Sanitaria Provinciale, Ragusa, Italy.
authors.forename: Maurizio
authors.initials: M
authors.lastname: Aricò
authors.affiliation: Istituto di Ricerca Pediatrica, Fondazione Città della Speranza, Padova, Italy.
authors.forename: Lara
authors.initials: L
authors.lastname: Mussolin
authors.affiliation: Department of Women's and Children's Health, Clinic of Pediatric Hemato-Oncology, University of Padova, Italy.
authors.forename: Elisa
authors.initials: E
authors.lastname: Carraro
authors.affiliation: Pediatric Oncology Department, Santobono-Pausilipon Hospital of Napoli, Italy.
authors.forename: Salvatore
authors.initials: S
authors.lastname: Buffardi
authors.affiliation: Department of Pediatrics, Division of Pediatric Hematology-Oncology, Bari, Italy.
authors.forename: Nicola
authors.initials: N
authors.lastname: Santoro
authors.affiliation: Department of Oncology, Pediatric Hematology and Oncology Unit, A.R.N.A.S. Ospedali Civico Di Cristina e Benfratelli, Palermo, Italy.
authors.forename: Paolo
authors.initials: P
authors.lastname: D'Angelo
authors.affiliation: Dipartimento di Onco-Ematologia Pediatrica, Ospedale Bambino Gesù, Roma, Italy.
authors.forename: Alessandra
authors.initials: A
authors.lastname: Lombardi
authors.affiliation: Division of Pediatric Hematology and Oncology, Ospedale G.Salesi, Ancona, Italy.
authors.forename: Paolo
authors.initials: P
authors.lastname: Pierani
authors.affiliation: Department of Pediatrics, Ospedale Papa Giovanni XXIII, Bergamo, Italy.
authors.forename: Eugenia
authors.initials: E
authors.lastname: Giraldi
authors.affiliation: Pediatric Hematology-Oncology, Ospedale Pediatrico Microcitemico, Cagliari, Italy.
authors.forename: Rossella
authors.initials: R
authors.lastname: Mura
authors.affiliation: Clinica Pediatrica, Università degli Studi di Milano-Bicocca, Azienda Ospedaliera San Gerardo, Monza, Italy.
authors.forename: Alessandra
authors.initials: A
authors.lastname: Sala
authors.affiliation: Department of Hematology-Oncology, Istituto Giannina Gaslini, Genova, Italy.
authors.forename: Alberto
authors.initials: A
authors.lastname: Garaventa
authors.affiliation: Department of Pediatric Hematology-Oncology, Azienda Ospedaliero-Universitaria Meyer Children Hospital, Firenze, Italy.
authors.forename: Annalisa
authors.initials: A
authors.lastname: Tondo
authors.affiliation: Division of Pediatric Onco-Hematology, Regina Margherita Children's Hospital, Torino, Italy.
authors.forename: Matilde
authors.initials: M
authors.lastname: Piglione
authors.affiliation: Pediatric Hematology-Oncology, Policlinico di Catania, Italy.
authors.forename: Luca
authors.initials: L
authors.lastname: Lo Nigro
authors.affiliation: Pediatric Hematology-Oncology, Azienda Ospedaliera Universitaria Integrata Verona, Italy.
authors.forename: Simone
authors.initials: S
authors.lastname: Cesaro
authors.affiliation: Oncoematologia Pediatrica, Azienda Ospedaliera-Universitaria di Perugia, Italy.
authors.forename: Katia
authors.initials: K
authors.lastname: Perruccio
authors.affiliation: Department of Women's and Children's Health, Clinic of Pediatric Hemato-Oncology, University of Padova, Italy.
authors.forename: Angelo
authors.initials: A
authors.lastname: Rosolen
authors.affiliation: Department of Women's and Children's Health, Clinic of Pediatric Hemato-Oncology, University of Padova, Italy.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Basso
authors.affiliation: Department of Women's and Children's Health, Clinic of Pediatric Hemato-Oncology, University of Padova, Italy.
authors.forename: Marta
authors.initials: M
authors.lastname: Pillon
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D030342:Genetic Diseases, Inborn / Q000150:complications*; D006801:Humans; D008228:Lymphoma, Non-Hodgkin / Q000150:complications* / Q000453:epidemiology*; D008297:Male; D012189:Retrospective Studies
keywords: associated genetic condition; ataxia-telangiectasia; childhood; non-Hodgkin lymphoma; treatment