Primary immunodeficiency disorders: antibody deficiency

Abstract

As a group, antibody deficiencies represent the most common types of primary immune deficiencies in human subjects. Often symptoms do not appear until the latter part of the first year of life, as passively acquired IgG from the mother decreases to below protective levels. As with the T-cell immune deficiencies, the spectrum of antibody deficiencies is broad, ranging from the most severe type of antibody deficiency with totally absent B cells and serum Igs to patients who have a selective antibody deficiency with normal serum Ig. In addition to the increased susceptibility to infections, a number of other disease processes (eg, autoimmunity and malignancies) can be involved in the clinical presentation. Fortunately, the availability of intravenous immune serum globulin has made the management of these patients more complete. Recently, molecular immunology has led to identification of the gene or genes involved in many of these antibody deficiencies. As discussed in this review, this has led to a better elucidation of the B-cell development and differentiation pathways and a more complete understanding of the pathogenesis of many of these antibody deficiencies.