Non-Hodgkin lymphoma in pediatric patients with common variable immunodeficiency

• Published in: European journal of pediatrics Vol. 174; no. 8; pp. 1069 - 1076
• Main Authors: Piquer Gibert, Monica, Alsina, Laia, Giner Muñoz, María Teresa, Cruz Martínez, Ofelia, Ruiz Echevarria, Karen, Dominguez, Olga, Plaza Martín, Ana María, Arostegui, Juan I., de Valles, Guillem, Juan Otero, Manel, Martin-Mateos, Maria Anunciacion
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer Berlin Heidelberg 01-08-2015; Springer Nature B.V
• Subjects: Adolescent; Astrocytoma - diagnosis; Astrocytoma - etiology; Astrocytoma - immunology; Bacterial infections; Child; Common Variable Immunodeficiency - complications; Common Variable Immunodeficiency - immunology; Disease; Early Diagnosis; Epstein-Barr virus; Female; Genotype & phenotype; Humans; Immune system; Immunoglobulins; Immunology; Incidence; Infections; Lymphoma; Lymphoma, Non-Hodgkin - diagnosis; Lymphoma, Non-Hodgkin - etiology; Lymphoma, Non-Hodgkin - immunology; Male; Medicine; Medicine & Public Health; Original Article; Patients; Pediatrics; Phenotype; Tumors; Spain; Rituximab; Epstein-Barr virus; Common variable immunodeficiency; MALT lymphoma; Second neoplasm; Pediatric
• ISSN: 0340-6199; 1432-1076
• DOI: 10.1007/s00431-015-2508-6

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.
What is Known:
• Non-Hodgkin lymphomas are the most frequent neoplasm reported in pediatric CVID patients.
• “Polyclonal lymphoproliferation” clinical phenotype is associated with increased risk of lymphoid malignancy and group smB-Tr
hi
of the Euroclass classification with an increased risk of lymphadenopathy
.
What is New:
•
We report a higher incidence of non-Hodgkin lymphomas compared to previous publications in pediatric patients, and our patients are younger than reported.
•
None of our patients belongs to “polyclonal lymphoproliferation” clinical phenotype, and a common B cell subphenotyping (smB+21
lo
) was identified in two of lo the three patients.