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Review
. 2011 Jun;170(6):693-702.
doi: 10.1007/s00431-011-1474-x. Epub 2011 May 5.

Educational paper: primary antibody deficiencies

Gertjan Driessen  1 , Mirjam van der Burg
Affiliations
Review

Educational paper: primary antibody deficiencies

Gertjan Driessen et al. Eur J Pediatr. 2011 Jun.

Abstract

Primary antibody deficiencies (PADs) are the most common primary immunodeficiencies and are characterized by a defect in the production of normal amounts of antigen-specific antibodies. PADs represent a heterogeneous spectrum of conditions, ranging from often asymptomatic selective IgA and IgG subclass deficiencies to the severe congenital agammaglobulinemias, in which the antibody production of all immunoglobulin isotypes is severely decreased. Apart from recurrent respiratory tract infections, PADs are associated with a wide range of other clinical complications. This review will describe the pathophysiology, diagnosis, and treatment of the different PADs.

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Figures

Fig. 1
Fig. 1
Antigen(Ag)-independent B cell differentiation occurs in the bone marrow, whereas Ag-dependent B cell differentiation occurs in the periphery. After activation by Ag, B cells develop in a T cell-dependent way in the germinal center and in a T cell-independent way in the marginal zone of the spleen. Defects of survival, Ag stimulation, B–T interaction, TI response, CSR/SHM can result in PAD. Identified PAD genes affecting these processes are indicated
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