Immunobiology of Primary Antibody Deficiencies: Towards a new classification

Primary antibody deficiencies (PADs) are the most common primary immunodeficiencies. The hallmark of PADs is a defect in the production of normal amounts of antigen specific antibodies. These antibodies or immunoglobulins are indispensible for the adaptive immune response against a wide variety of pathogens. A defect in antibody production results in recurrent and/or severe infections. PADs represent a heterogeneous spectrum of conditions, ranging from often asymptomatic selective IgA and IgG subclass deficiencies to the severe congenital agammaglobulinemia’s, in which antibody production of all immunoglobulin isotypes is severely decreased. Apart from recurrent infections there is a wide range of other clinical complications associated with primary antibody deficiency, affecting quality of life and life expectancy. Primary antibody deficiencies are the result of primary or secondary defects in B-cell development. This Chapter will discuss the principles of adaptive immunity including normal B-cell development, followed by an introduction of the known immunogenetic and clinical characteristics of primary antibody deficiency. Next, we give an overview of potential pathophysiological mechanism in idiopathic primary immunodeficiency. Finally, the aims of the thesis are explained.

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