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Published on: September 7, 2022
Age-related alterations of the CD19 complex and memory B cells in children with Down syndrome
Ayse Nazli Seckin1, Hulya Ozdemir1, Ayca Ceylan1
1Department of Pediatric Immunology and Allergy, Selcuk University Medical Faculty, Alaeddin Keykubat Kampusu, 42131, Konya, Turkey.
Insights
Children with Down syndrome (DS) exhibit immune dysfunction, characterized by lower B cell counts and altered CD19, CD21, and CD81 expression. These B cell defects may explain their increased susceptibility to infections.
Area of Science:
- Immunology
- Pediatrics
Background:
- Children with Down syndrome (DS) frequently experience recurrent respiratory infections, leukemia, and autoimmune disorders, indicating underlying immune system dysfunction.
- The specific immunological defects contributing to this vulnerability are not fully understood, particularly concerning B cell populations.
Purpose of the Study:
- To investigate the role of the CD19 complex and memory B cells in the immunodeficiency observed in children with Down syndrome.
- To analyze the expression levels of key B cell surface molecules (CD19, CD21, CD81) and memory B cell subsets in children with DS.
Main Methods:
- Flow cytometry was used to measure the expression of CD19, CD21, and CD81 on B cells and memory B cell subsets.
- A cohort of 37 children with DS (29 with congenital heart disease) and 39 healthy controls were studied across different age groups.
Main Results:
- Children with DS showed significantly lower B cell counts across all age groups compared to controls.
- Reduced CD19 expression was observed in all age groups, while CD21 expression increased in those over 2 years, and CD81 expression increased in those over 6 years.
- A reduced frequency of natural effector B cells (CD27+IgD+IgM+) was found in children with DS hospitalized for infections.
Conclusions:
- Intrinsic defects in B cells, including altered cell counts and surface molecule expression, are present in children with Down syndrome.
- These B cell abnormalities likely contribute to the increased susceptibility to severe respiratory tract infections in this population.
Abstract:
Children with Down syndrome (DS) have a high incidence of recurrent respiratory tract infections, leukaemia and autoimmune disorders, suggesting immune dysfunction. The present study evaluated the role of the CD19 complex and memory B cells in the pathogenesis of immunodeficiency in children with DS. The expression levels (median fluorescein intensity-MFI) of CD19, CD21 and CD81 molecules on the surface of B cells and memory B cell subsets were studied in 37 patients and 39 healthy controls. Twenty-nine of the DS group had congenital cardiac disease. The B cell count was significantly low in children with DS compared with healthy age-matched controls for all three age groups (under 2 years; 2-6 years and older than 6 years). The MFI of CD19 was reduced in all the age groups, whereas that of CD21 was increased in those older than 2 years with DS. The expression level of CD81 was significantly increased in those older than 6 years. Age-related changes were also detected in memory B cell subsets. The frequency of CD27+IgD+IgM+ natural effector B cells was reduced in children with DS who had needed hospitalisation admission due to infections. The observed intrinsic defects in B cells may be responsible for the increased susceptibility of children with DS to severe respiratory tract infections.
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