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Primary immune deficiencies affecting lymphocyte differentiation: lessons from the spectrum of resulting infections


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 T(h)17 T cells. Together with defects specific to T(h)1 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 T(h)17 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.

Type Journal
ISBN 1460-2377 (Electronic)
Authors Cook, M. C.; Tangye, S. G.;
Garvan Authors Prof Stuart Tangye
Publisher Name INT IMMUNOL
Published Date 2009-09-01 00:00:00
Published Volume 21
Published Issue 9
Published Pages 1003-11
Status Published in-print