Non-hodgkin lymphoma in children with an associated inherited condition: A retrospective analysis of the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP)

Maurizio Aricò, Lara Mussolin, Elisa Carraro, Salvatore Buffardi, Nicola Santoro, Paolo D'Angelo, Alessandra Lombardi, Paolo Pierani, Eugenia Giraldi, Rossella Mura, Alessandra Sala, Alberto Garaventa, Annalisa Tondo, Matilde Piglione, Luca Lo Nigro, Simone Cesaro, Katia Perruccio, Angelo Rosolen, Giuseppe Basso, Marta Pillon

Research output: Contribution to journalArticle

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). Conclusion: 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.

Original languageEnglish
Pages (from-to)1782-1789
Number of pages8
JournalPediatric Blood and Cancer
Volume62
Issue number10
DOIs
Publication statusPublished - Oct 1 2015

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Keywords

  • Associated genetic condition
  • Ataxia-telangiectasia
  • Childhood
  • Non-Hodgkin lymphoma
  • Treatment

ASJC Scopus subject areas

  • Oncology
  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Medicine(all)

Cite this

Aricò, M., Mussolin, L., Carraro, E., Buffardi, S., Santoro, N., D'Angelo, P., Lombardi, A., Pierani, P., Giraldi, E., Mura, R., Sala, A., Garaventa, A., Tondo, A., Piglione, M., Lo Nigro, L., Cesaro, S., Perruccio, K., Rosolen, A., Basso, G., & Pillon, M. (2015). Non-hodgkin lymphoma in children with an associated inherited condition: A retrospective analysis of the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP). Pediatric Blood and Cancer, 62(10), 1782-1789. https://doi.org/10.1002/pbc.25565