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- Thomas A. Fleisher, MD*
- *Chief, Department of Laboratory Medicine, Clinical Center, National Institutes of Health, Department of Health and Human Services, Bethesda, Md
Objectives
After completing this article, readers should be able to:
Describe the basic role of the innate and adaptive immune systems in host defense.
Recognize the clinical features of the various categories of primary immune deficiencies.
List the screening laboratory tests that can define the primary immune deficiencies.
Discuss the general approaches to immune reconstitution for treating primary immune deficiencies.
Introduction
The inherited immune deficiencies have been invaluable instructors, extending our understanding of the contribution of components of the immune system to host defense. The information provided by studying these disorders also has generated new avenues of therapy, applications of which have influenced the treatment of a range of other human diseases. Currently, more than 120 genetic defects are associated with immune deficiency, and it appears that these numbers will continue to grow. This expansion of knowledge is being driven, in part, by clinicians becoming more aware of the subtleties of immune function and linking clinical phenotypes to potential immune deficiencies. This article focuses on the range of human immune deficiencies in the context of the underlying immunologic defects and associated infections. This link between components of the immune system and protection from infection to various categories of microorganisms serves as the basis for developing current approaches to therapy. Space constraints limit this discussion; readers are referred to major textbooks that deal specifically with immune deficiencies for in-depth discussions of these disorders. (1)
Better understanding of the immune deficiencies requires a basic understanding of the immune system and its division into two major components: innate and adaptive immunity. The former is a phylogenetically more primitive system that responds without the requirement of prior exposure. The functional components of innate immunity include cellular components as well as circulating and secreted proteins (Fig. 1). An innate immune host response is activated by direct …
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