International Immunology Advance Access originally published online on August 3, 2009
International Immunology 2009 21(9):1003-1011; doi:10.1093/intimm/dxp076
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review-article |
Primary immune deficiencies affecting lymphocyte differentiation: lessons from the spectrum of resulting infections
1 John Curtin School of Medical Research, Australian National University, Canberra 2600, Australia
2 Department of Immunology, The Canberra Hospital, PO Box 11, Woden, ACT 2606, Australia
3 Immunology and Inflammation Program, Garvan Institute of Medical Research, Darlinghurst, Australia
4 St Vincent's Clinical School, University of New South Wales, Kensington, Australia
Correspondence to: M. C. Cook; E-mail: matthew.cook{at}anu.edu.au
Understanding primary immunodeficiencies has elucidated many aspects of human immunity and susceptibility to infections. Recently, defects have been identified that result in deficiencies of terminally differentiated subsets of lymphocytes including deficiencies of memory B cells, NKT cells and Th17 T cells. Together with defects specific to Th1 responses, these disorders revealed that dedicated pathogen-specific mechanisms exist for prevalent human pathogens, and that some host defence strategies are remarkably specific. Deficiency of Th17 cells confirms that this subset of effector T cells is important for defence at epithelial surfaces. The clinical phenotype includes devastating complications from infection with Staphylococcus aureus. Since the microbial load at human epithelial surfaces is substantial and enormously diverse, this specificity could hold clues that are important for understanding first the complex symbiosis with mucosal commensals and second for understanding the consequences of manipulating these populations in inflammatory diseases.
Keywords: immunodeficiencies, infectious susceptibility, lymphocyte differentiation
Received 22 February 2009, accepted 15 July 2009.