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Original Article


B-Cell Subsets in Patients With Transient Hypogammaglobulinemia of Infancy, Partial IgA Deficiency, and Selective IgM Deficiency


FE Çipe,1 F Doğu,1 D Güloğlu,1 C Aytekin,1 M Polat,1 Z Bıyıkli,2 A Ikincioğulları1

1 Department of Pediatric Immunology-Allergy, Ankara University School of Medicine, Ankara, Turkey
2 Department of Biostatistics, Ankara University School of Medicine, Ankara, Turkey

J Investig Allergol Clin Immunol 2013; Vol. 23(2): 94-100



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.

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.

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.

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.

Key words:
Hypogammaglobulinemia. IgA deficiency. IgM deficiency. Memory B cell.