Ismail Ogulur1,2, Ayca Kiykim1, Dilek Baser1, Elif Karakoc-Aydiner1,2, Ahmet Ozen1,2, Safa Baris3,4. 1. Division of Pediatric Allergy-Immunology, Faculty of Medicine, Marmara University, Istanbul, Turkey. 2. Istanbul Jeffrey Modell Diagnostic and Research Center for Primary Immunodeficiencies, Istanbul, Turkey. 3. Division of Pediatric Allergy-Immunology, Faculty of Medicine, Marmara University, Istanbul, Turkey, safabaris@hotmail.com. 4. Istanbul Jeffrey Modell Diagnostic and Research Center for Primary Immunodeficiencies, Istanbul, Turkey, safabaris@hotmail.com.
Abstract
INTRODUCTION: Common variable immunodeficiency (CVID) is characterized by recurrent infections, autoimmunity, lymphoproliferation, hypogammaglobulinemia, and defective antibody production. In CVID, B-cell abnormalities were described to predict end organ involvement and prognosis. Pediatric-onset CVID is much rarer than adult CVID, and lymphocyte subset abnormalities have not been thoroughly evaluated. OBJECTIVE: We sought to determine lymphocyte subset abnormalities and their association with end organ involvement in pediatric-onset CVID patients. METHODS: The clinical manifestations and laboratory findings including absolute numbers and percentages of B-, T-, and NK cell populations were assessed in pediatric-onset CVID patients and compared to age-matched healthy controls. The patients were divided into 2 groups according to age at assessment (pediatric CVID patients: 10-16 years, n = 9; and adult CVID patients: >16 years, n = 13). The comparisons between lymphocyte subsets and organ involvement were also evaluated. RESULTS: Mean age at symptom onset was 18 (3-204) months. All CVID patients with pediatric onset had decreased levels of total and memory B cells, CD4+ T cells, CD4+CD45RA+ naive T cells, and recent thymic emigrant (RTE) cells. On the other hand, they had increases in CD8+CD45RO+ memory T cells. Interestingly, adult CVID patients demonstrated high frequencies of activated and double-negative T cells, which were unique only for this group of patients. Specific cellular abnormalities associated with the reduction in B and NK cells and increase in CD8+ T cells were found in patients with bronchiectasis. Moreover, in pediatric CVID patients, low serum IgA levels and decreased numbers of naive T and RTE cells were determined as risk factors for chronic diarrhea. CONCLUSIONS: Specific abnormalities in B- and T-lymphocyte compartments were identified in pediatric-onset CVID patients and appear to be associated with end organ manifestations.
INTRODUCTION:Common variable immunodeficiency (CVID) is characterized by recurrent infections, autoimmunity, lymphoproliferation, hypogammaglobulinemia, and defective antibody production. In CVID, B-cell abnormalities were described to predict end organ involvement and prognosis. Pediatric-onset CVID is much rarer than adult CVID, and lymphocyte subset abnormalities have not been thoroughly evaluated. OBJECTIVE: We sought to determine lymphocyte subset abnormalities and their association with end organ involvement in pediatric-onset CVIDpatients. METHODS: The clinical manifestations and laboratory findings including absolute numbers and percentages of B-, T-, and NK cell populations were assessed in pediatric-onset CVIDpatients and compared to age-matched healthy controls. The patients were divided into 2 groups according to age at assessment (pediatric CVIDpatients: 10-16 years, n = 9; and adult CVIDpatients: >16 years, n = 13). The comparisons between lymphocyte subsets and organ involvement were also evaluated. RESULTS: Mean age at symptom onset was 18 (3-204) months. All CVIDpatients with pediatric onset had decreased levels of total and memory B cells, CD4+ T cells, CD4+CD45RA+ naive T cells, and recent thymic emigrant (RTE) cells. On the other hand, they had increases in CD8+CD45RO+ memory T cells. Interestingly, adult CVIDpatients demonstrated high frequencies of activated and double-negative T cells, which were unique only for this group of patients. Specific cellular abnormalities associated with the reduction in B and NK cells and increase in CD8+ T cells were found in patients with bronchiectasis. Moreover, in pediatric CVIDpatients, low serum IgA levels and decreased numbers of naive T and RTE cells were determined as risk factors for chronic diarrhea. CONCLUSIONS: Specific abnormalities in B- and T-lymphocyte compartments were identified in pediatric-onset CVIDpatients and appear to be associated with end organ manifestations.
Authors: Aleksandra Szczawińska-Popłonyk; Katarzyna Ta Polska-Jóźwiak; Eyal Schwartzmann; Natalia Popłonyk Journal: Front Pediatr Date: 2022-03-23 Impact factor: 3.418
Authors: Francesco Rispoli; Erica Valencic; Martina Girardelli; Alessia Pin; Alessandra Tesser; Elisa Piscianz; Valentina Boz; Flavio Faletra; Giovanni Maria Severini; Andrea Taddio; Alberto Tommasini Journal: Diagnostics (Basel) Date: 2021-03-16