Primary Antibody Deficiencies

  • M. D. Cooper
  • A. R. Lawton
  • J. L. Preud’homme
  • M. Seligmann
Chapter

Abstract

It is increasingly apparent that defective antibody responses can be the common underlying basis for a wide variety of diseases formerly thought to involve the immune system only secondarily. In a large measure information leading to this awareness has been obtained from experimental dissection of the multiple components of the immune system, and analysis of how they develop and interact in the normal immune response to exogenous antigens and altered self antigens. Accurate methods for identifying different cell types involved in host defense and the functional products that they make at different stages during differentiation are now available for the clinical investigation of immune system dysfunction. In this chapter, we will first present a condensed description of the ontogeny and continual differentiation of the B cell lineage. This conceptual framework will then be used to discuss the pathogenesis of different antibody deficiency syndromes. It is our hope to illustrate the usefulness of this way of analyzing disorders of the immune system. More importantly, this discussion should serve to emphasize gaps in knowledge of genetic and molecular aspects of immune function which need filling before corrective therapy can be devised for many immunodeficient patients.

Keywords

Major histocompatibIlity Complex Severe Combine Immunodeficiency Primary Immunodeficiency Disease Antibody Deficiency Pokeweed Mitogen 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
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Copyright information

© Springer-Verlag Berlin Heidelberg 1979

Authors and Affiliations

  • M. D. Cooper
    • 1
  • A. R. Lawton
    • 1
  • J. L. Preud’homme
    • 2
  • M. Seligmann
    • 2
  1. 1.Cellular Immunobiology Unit of the Tumor Institute, Departments of Pediatrics and Microbiology and the Comprehensive Cancer CenterUniversity of Alabama in BirminghamBirminghamUSA
  2. 2.Laboratoire d’Immunochimie et d’Immunopathologie (INSERM U. 108), Institute de Recherches sur les Maladies du SangHôpital Saint-LouisParis 10France

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