Publication:
Lymphocyte Subset Abnormalities in Pediatric-Onset Common Variable Immunodeficiency

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dc.contributor.authorÖZEN, AHMET OĞUZHAN
dc.contributor.authorsOgulur, Ismail; Kiykim, Ayca; Baser, Dilek; Karakoc-Aydiner, Elif; Ozen, Ahmet; Baris, Safa
dc.date.accessioned2022-03-12T22:41:01Z
dc.date.available2022-03-12T22:41:01Z
dc.date.issued2020
dc.description.abstractIntroduction: 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.
dc.identifier.doi10.1159/000504598
dc.identifier.eissn1423-0097
dc.identifier.issn1018-2438
dc.identifier.pubmed31901904
dc.identifier.urihttps://hdl.handle.net/11424/236055
dc.identifier.wosWOS:000518606900008
dc.language.isoeng
dc.publisherKARGER
dc.relation.ispartofINTERNATIONAL ARCHIVES OF ALLERGY AND IMMUNOLOGY
dc.rightsinfo:eu-repo/semantics/closedAccess
dc.subjectCommon variable immunodeficiency
dc.subjectPediatric patients
dc.subjectLymphocyte subsets
dc.subjectEnd organ involvement
dc.subjectB-CELL
dc.subjectDEFICIENCY
dc.subjectDIFFERENTIATION
dc.subjectCLASSIFICATION
dc.subjectSUBGROUPS
dc.subjectCHILDREN
dc.titleLymphocyte Subset Abnormalities in Pediatric-Onset Common Variable Immunodeficiency
dc.typearticle
dspace.entity.typePublication
local.avesis.id85616445-a8ee-46e0-ae05-51a9fc732c98
local.import.packageSS17
local.indexed.atWOS
local.indexed.atSCOPUS
local.indexed.atPUBMED
local.journal.numberofpages10
local.journal.quartileQ3
oaire.citation.endPage237
oaire.citation.issue3
oaire.citation.startPage228
oaire.citation.titleINTERNATIONAL ARCHIVES OF ALLERGY AND IMMUNOLOGY
oaire.citation.volume181
relation.isAuthorOfPublication3e9c297b-e636-4836-8f61-dc9c8b7c29cf
relation.isAuthorOfPublication.latestForDiscovery3e9c297b-e636-4836-8f61-dc9c8b7c29cf

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