B-cell subsets in patients with transient hypogammaglobulinemia of infancy, partial IgA deficiency, and selective IgM deficiency.

Abstract

BACKGROUND:

The pathogenesis of some primary humoral immunodeficiencies, such as transient hypogammaglobulinemia of infancy (THI) and immunoglobulin (Ig) A deficiency, remains unknown and can render diagnosis problematic.

OBJECTIVE:

In the present study, we used flow cytometry to analyze peripheral blood B-cell subsets in patients with THI and unclassified hypogammaglobulinemia (UCH), partial IgA deficiency, and selective IgM deficiency.

METHODS:

The study population comprised 41 patients with hypogammaglobulinemia (THI, 18; UCH, 23), 16 patients with partial IgA deficiency, and 16 patients with selective IgM deficiency who were admitted to Ankara University Department of Pediatric Immunology-Allergy between January 2010 and April 2011, as well as 29 healthy controls. B-cell subsets were examined according to the EUROclass classification.

RESULTS:

Age at diagnosis in the hypogammaglobulinemia group ranged between-14 months and 13 years (median, 26 months). Naive B-cell percentages were significantly higher and activated B-cell values lower in the THI patients than in the UCH patients and age-matched healthy controls. Nonswitched (IgM+CD27+IgD+) memory B-cell values were found to be significantly lower in patients with selective IgM deficiency than in healthy controls. No significant differences in B-cell subsets were found in patients with partial IgA deficiency.

CONCLUSIONS:

Previous reports show that reduced class-switched memory B cell values are associated with CVID, THI, and selective IgA deficiency. Our findings did not support these reports. Furthermore, we observed that naive B cell values were higher in patients with THI. A maturation defect could play a role in the pathogenesis of THI.

• Authors: F E Cipe, F Doğu, D Güloğlu, C Aytekin, M Polat, Z Biyikli, A Ikincioğullari
• Journal: Journal of investigational allergology & clinical immunology (J Investig Allergol Clin Immunol) Vol. 23 Issue 2 Pg. 94-100 ( 2013) ISSN: 1018-9068 [Print] Spain
• PMID: 23654075 (Publication Type: Journal Article)

Chemical References

• Immunoglobulin A
• Immunoglobulin D
• Immunoglobulin M

Topics

• Adolescent
• Agammaglobulinemia (genetics, immunology, pathology)
• B-Lymphocyte Subsets (immunology, pathology)
• Case-Control Studies
• Child
• Child, Preschool
• Female
• Flow Cytometry
• Gene Expression
• Humans
• IgA Deficiency (genetics, immunology, pathology)
• Immunoglobulin A (genetics, immunology)
• Immunoglobulin Class Switching
• Immunoglobulin D (genetics, immunology)
• Immunoglobulin M (deficiency, genetics, immunology)
• Immunologic Memory
• Infant
• Male