Non-Hodgkin lymphoma in pediatric patients with common variable immunodeficiency.
Common variable immunodeficiency (CVID) is a heterogeneous primary immunodeficiency associated with an increased risk of malignancy in adulthood, with lymphoma as one of the major causes of death. The aim of this study is to describe those malignancies detected in our cohort of pediatric CVID patients. We reviewed the clinical and laboratory data and the treatments and their outcomes in all pediatric CVID patients from our institution that developed a neoplasia. Four malignancies were diagnosed in three out of 27 pediatric CVID patients. Three malignancies were non-Hodgkin lymphoma (NHL) of B cell origin (mean age at diagnosis: 8 years old), and the remaining was a low-grade astrocytoma. Among NHL, two were mucosa-associated lymphoid tissue (MALT) lymphomas and one was associated with Epstein-Barr virus infection. NHL developed before CVID diagnosis in two patients. CVID patients showed different clinical phenotypes and belonged to different groups according Euroclass and Pediatric classification criteria.


CONCLUSIONS
Malignancies, especially lymphoma, may develop in pediatric CVID patients with no previous signs of lymphoid hyperplasia and even before CVID diagnosis. Consequently, strategies for cancer prevention and/or early diagnosis are required in pediatric CVID patients.
Pediatric Allergy and Clinical Immunology Department, Hospital Sant Joan de Déu-Universitat de Barcelona, Passeig Sant Joan de Déu, 2, 08950, Esplugues de Llobregat, Barcelona, Spain, mpiquer@hsjdbcn.org.
Monica
M
Piquer Gibert
Laia
L
Alsina
María Teresa
MT
Giner Muñoz
Ofelia
O
Cruz Martínez
Karen
K
Ruiz Echevarria
Olga
O
Dominguez
Ana María
AM
Plaza Martín
Juan I
JI
Arostegui
Guillem
G
de Valles
Manel
M
Juan Otero
Maria Anunciacion
MA
Martin-Mateos
D000293:Adolescent; D001254:Astrocytoma / Q000175:diagnosis* / Q000209:etiology / Q000276:immunology; D002648:Child; D017074:Common Variable Immunodeficiency / Q000150:complications* / Q000276:immunology; D042241:Early Diagnosis; D005260:Female; D006801:Humans; D015994:Incidence; D008228:Lymphoma, Non-Hodgkin / Q000175:diagnosis* / Q000209:etiology / Q000276:immunology; D008297:Male; D010641:Phenotype