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

Date

2009

Authors

Cook, Matthew
Tangye, Stuart G

Journal Title

Journal ISSN

Volume Title

Publisher

Oxford University Press

Abstract

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.

Description

Keywords

Keywords: antigen presentation; B lymphocyte; cell damage; cell surface; cellular immunity; commensal; epithelium cell; host pathogen interaction; host resistance; human; immune deficiency; infection sensitivity; infectious complication; lymphocyte differentiation; Immunodeficiencies; Infectious susceptibility; Lymphocyte differentiation

Citation

Source

International Immunology

Type

Journal article

Book Title

Entity type

Access Statement

License Rights

Restricted until

2037-12-31