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Allergologia et Immunopathologia
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Inicio Allergologia et Immunopathologia Recent thymic emigrants, T regulatory cells, and BAFF level in children with X-l...
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Vol. 46. Num. 1.January - February 2018
Pages 1-104
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Vol. 46. Num. 1.January - February 2018
Pages 1-104
Original Article
DOI: 10.1016/j.aller.2017.01.011
Recent thymic emigrants, T regulatory cells, and BAFF level in children with X-linked agammaglobulinaemia in association with chronic respiratory disease
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S.O. Sharapovaa,
Corresponding author
sharapovasv@gmail.com

Corresponding author.
, O.E. Pashchenkob, I.E. Guryanovaa, A.A. Migasa, I.V. Kondratenkob, O.V. Aleinikovaa
a Research Department, Belarusian Research Center for Pediatric Oncology, Hematology and Immunology, Minsk Region, Belarus
b Department of Clinical Immunology, Russian Clinical Children's Hospital, Moscow, Russia
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Table 1. Clinical and genetic characteristic of XLA patients. Mutations of patients, ages at diagnosis, ages of blood investigation and co-morbidities.
Abstract
Background

X-linked agammaglobulinaemia (XLA) is a genetic disorder affecting B cell maturation, which is characterised by a low number of B cells, agammaglobulinaemia and increased susceptibility to a variety of bacterial infections. This study was performed to assess T cell subpopulations in a group of children with XLA in association with chronic respiratory disease (CRD).

Methods

Numbers of T cell subpopulations (CD3+, CD4+, CD8+, CD3+DR+, naïve, memory, recent thymic emigrants (RTE), regulatory T cells, follicular T helpers) were measured by eight-colour flow cytometry in 22 XLA patients and 50 controls. BAFF level was measured by ELISA.

Results

XLA patients with CRD had a significantly lower percentage of RTE numbers and Tregs, while significantly higher absolute counts of lymphocytes, CD3+, CD8+, CD3+DR+ and CD4+CD45RO+ T cells were detected as compared with healthy controls. In patients with XLA without CRD, the number of follicular T helper cells was altered significantly (percentage and absolute), as compared with healthy controls. Additionally, they had significantly higher counts (percentage and absolute) of CD4+CD45RA+ cells and lower percentage of CD4+CD45RO+ cells in comparison with healthy controls.

Conclusions

Our study affords new information concerning CRD and T cell subsets that differentiate or are maintained in the absence of B cells in children with XLA. T cell's homeostasis depends on the presence of chronic respiratory disease that may be caused by the delay in diagnosis.

Keywords:
X-linked agammaglobulinaemia
T cells
BAFF
Chronic lung infections
Chronic sinusitis

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