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Background  Hypogammaglobulinemia in early childhood is a common feature characterized by distinct intrinsic and extrinsic factors leading to disturbed peripheral blood lymphocyte homeostasis. Detailed flow cytometric immunophenotyping of the peripheral blood B cell compartment is an informative tool for delineating disturbed generation of B cell subpopulations crucial for the diagnosis of hypogammaglobulinemia in young children.    Methods  We analyzed by flow cytometry the proportions and absolute values of total, naïve, memory - non-switched and switched, transitional and immature B lymph cells as well as plasmablasts in the peripheral blood of 50 hypogammaglobulinemic children aged from 3 to 50 months.    Results  Beyond physiological, age-related changes within the B cell pool, a proportion of children manifested defective differentiation into switched memory and accumulation of CD21lo immature B cells.    Conclusions  Dynamic shifts within B cell subpopulations of the immature immune system being most prominent during the first two years of life contribute to the age-related developmental abnormalities of the B cell compartment. Therefore, a reliable diagnosis of common variable immunodeficiency (CVID) in young hypogammaglobulinemic children cannot yet be established despite their clinical and immunological phenotypes sharing common features with this primary immunodeficiency.

The B-cell compartment in antibody-deficient infants and young children – developing common variable immunodeficiency or transient immune maturation?