Biochemical Defects of Polymorphonuclear and Mononuclear Phagocytes Associated with Disease

Abstract

As its title indicates, the intent of this chapter is to review the known biochemical defects of human neutrophils, monocytes, and macrophages that predispose to disease, in particular, to recurrent infections. The review will be organized along the lines of the principal functions of phagocytic cells in host defense— Chemotaxis, ingestion, and microbicidal activity—in an effort to place the biochemical abnormalities in the perspective of function and, thereby, to emphasize molecular -functional interactions wherever possible. The better substantiated disorders will be listed in tables, and those for which there exists some understanding of a molecular basis will be described in the text. When defects in more than one function coexist in the same disorder, somewhat arbitrary categorization will be made on the basis of apparent relative severity of the defects and the clinical pattern of infection. Since description of the clinical syndromes and functional abnormalities has greatly exceeded elucidation of their underlying molecular basis, this attempt to logically organize disorders of phagocyte function can be considered only tentative. Some description of the sites of infection and the infecting organisms will be included, in the hopes that integration of biochemical, functional, and clinical information might lend some insight into basic principles of leukocyte physiology and host defense against infection.