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

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. © 2013 esmon publicidad.