Format

Send to

Choose Destination
Pediatr Blood Cancer. 2015 Oct;62(10):1782-9. doi: 10.1002/pbc.25565. Epub 2015 May 22.

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

Author information

1
Azienda Sanitaria Provinciale, Ragusa, Italy.
2
Istituto di Ricerca Pediatrica, Fondazione Città della Speranza, Padova, Italy.
3
Department of Women's and Children's Health, Clinic of Pediatric Hemato-Oncology, University of Padova, Italy.
4
Pediatric Oncology Department, Santobono-Pausilipon Hospital of Napoli, Italy.
5
Department of Pediatrics, Division of Pediatric Hematology-Oncology, Bari, Italy.
6
Department of Oncology, Pediatric Hematology and Oncology Unit, A.R.N.A.S. Ospedali Civico Di Cristina e Benfratelli, Palermo, Italy.
7
Dipartimento di Onco-Ematologia Pediatrica, Ospedale Bambino Gesù, Roma, Italy.
8
Division of Pediatric Hematology and Oncology, Ospedale G.Salesi, Ancona, Italy.
9
Department of Pediatrics, Ospedale Papa Giovanni XXIII, Bergamo, Italy.
10
Pediatric Hematology-Oncology, Ospedale Pediatrico Microcitemico, Cagliari, Italy.
11
Clinica Pediatrica, Università degli Studi di Milano-Bicocca, Azienda Ospedaliera San Gerardo, Monza, Italy.
12
Department of Hematology-Oncology, Istituto Giannina Gaslini, Genova, Italy.
13
Department of Pediatric Hematology-Oncology, Azienda Ospedaliero-Universitaria Meyer Children Hospital, Firenze, Italy.
14
Division of Pediatric Onco-Hematology, Regina Margherita Children's Hospital, Torino, Italy.
15
Pediatric Hematology-Oncology, Policlinico di Catania, Italy.
16
Pediatric Hematology-Oncology, Azienda Ospedaliera Universitaria Integrata Verona, Italy.
17
Oncoematologia Pediatrica, Azienda Ospedaliera-Universitaria di Perugia, Italy.

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.

KEYWORDS:

associated genetic condition; ataxia-telangiectasia; childhood; non-Hodgkin lymphoma; treatment

PMID:
26011068
DOI:
10.1002/pbc.25565
[Indexed for MEDLINE]

Supplemental Content

Full text links

Icon for Wiley
Loading ...
Support Center