Abstract
Elie Metchnikoff [62] was the first to appreciate that circulating phagocytic cells protect the host from invading microbes and are essential for host survival. His observations, made almost 100 years ago, have been confirmed and extended in the laboratory and in the clinic. Today much is known about the complex interaction between humoral factors and phagocytic cells necessary for host defense against invading microbes.
P. G. Quie is the American Legion Memorial Heart Professor of Pediatrics
E. L. Mills is a Minnesota Heart Association Fellow
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Preview
Unable to display preview. Download preview PDF.
Similar content being viewed by others
References
Alexander, J. W., Hegg, M., Altmeier, W. A.: Neutrophil function in selected surgical disorders. Ann. Surg. 168, 447 (1968)
Altman, LC., Syndermann, R., Blaese, R. M.: Abnormalities of chemotactic lymphokinase synthesis and mononuclear leukocyte chemotaxis in Wiskott-Aldrich syndrome. J. Clin. Invest. 54, 486 (1974)
Anderson, R., Sher, R., Robson, A. R., Koornof, H. J.: Defective chemotaxis in measles patients. S. Africa Med. J. 48, 1819 (1974)
Arakawa, T., Wada, Y., Hayashi, T., Kakizaki, R., Chida, N., Chida, R., Kunno, T., Shioura, H.: Uracil-uric refractory anemia with peroxidase negative neutrophils. Tohoku J. Exp. Med. 87, 52 (1965)
Archer, G. T., Air, G., Jackas, M., Morell, D. M.: Studies on rat eosinophil peroxidase. Biochem. Biophys. Acta 99, 96 (1965)
Armstrong, D., Dimmitt, S., Boehme, D. H., S. C. Jr., Vogel, W.: Leukocyte peroxidase deficiency in a family with a dominant form of Kurs disease. Science 186, 155 (1974)
Armstrong, D., Dimmitt, S., Van Wormer, D. E.: Studies in Batten disease. I. Peroxidase deficiency in granulocytes. Arch. Neurol. 30, 144 ( 1974
Baehner, R. L., Karnovsky, M. L.: Deficiency of reduced nicotinamide-adenine dinclueotide oxidase in chronic granulomatous disease. Science 162, 1277 (1968)
Baehner, R. L., Johnston, R. B. Jr., Nathan, D. G.: Reduced pyridine nucleotide (RPN) content in G6PD-deficient granulocytes (PMN): an explanation for their defective bactericidal function. J. Clin. Invest. 50, 4a (1971)
Baehmer, R. L., Johnston, R. B. Jr., Nathan, D. G.: Comparative study of the metabolic and bactericidal characteristics of several glucose-6-phosphate dehydrogenase-deficient polymorphonuclear leukocytes and leukocytes from children with chronic granulomatous disease. J. Reticuloendothel. Soc. 12, 150 (1972)
Baehner, R. L., Neiburger, R. G., Johnston, D. E.: Bactericidal defect in blood of children with acute lymphoblastic leukemia. N. Engl. J. Med. 28, 1209 (1973)
Björksten, B., Kundmark, K. M.: Recurrent bacterial infections in four siblings with neutropenia, eosinophilia, hyperimmunoglobulinemia A, and defective neutrophil chemotaxis. J. Infect. Dis. 133, 63 (1976)
Blume, R.: Recurrent severe staphylococcal infections, eczematoid rash, extreme elevation of IgE, eosinophilia divergent chemotactic responses in two generations. J. Pediat, 90, 607 (1977)
Blume, R. S., Wolf, S. M.: The Chediak-Higashi syndrome: Studies in four patients and a review of the literature. Medicine 51, 247 (1972)
Boxer, L. A., Hedley-White, E. T., Stossel, T. P.: Neurophil actin dysfunction and abnormal neutrophil behavior. N. Engl. J. Med. 291, 1093 (1974)
Boxer, L. A., Watanabe, A. M., Rister, M., Besch, H. R., Allen, J., Beahner, R. L.: Correction of leukocyte function in Chediak-Higashi syndrome by ascorbate. N. Engl. J. Med. 295, 1041 (1976)
Boxer, L. A., Rister, M., Allen, J. M., Beahner, R. L.: Improvement of Chediak-Hidashi leukocyte function by cyclic guanosine monophosphate. Blood 49, 9 (1977)
Bullock, J. D., Robertson, A. F., Bodenbeuder, M. T., Kontras, S. B., Miller C. E.: Inflammatory response in the neonate re-examined. Pediatrics 44, 58 (1969)
Burge, P. S., Johnson, W. S., Hayward, A. R.: Neutrophil pyruvate kinase deficiency with recurrent staphylococcal infections: First reported case. Br. Med. L. 1, 742 (1976)
Clark, R. A., Kimball, H. R.: Defective granulocyte chemotaxis in the Chediak-Higashi syndrome. J. Clin. Invest. 50, 2645 (1974)
Clark, R. A., Root, R. K., Kimball, H. R., Kirkpatrick, C. H.: Defective neutrophil chemotaxis and cellular immunity in a child with recurrent infections. Ann. Intern. Med. 78, 515 (1973)
Cooper, M. R., DeChatelet, L. R., McCall, C. E., LaVia, M., Spurr, C. L., Baehner, R. L.: Leucocyte G-6-PD deficiency. Lancet 1970 II 110
Cooper, M. R., DeChatelet, L. R., McCall, C. E., LaVia, M. F., Spurr, C. L., Baehner, R. L.: Complete deficiency of leukocyte glucose-6-phosphate dehydrogenase with defective bactericidal activity. J. Clin. Invest. 51, 769 (1972)
Craddock, P. R., Yawata, Y., VanSanten, L., Gilberstadt, S., Silvis, S., Jacob, H. S.: Acquired phagocyte dysfunction: A complication of the hypophosphatemia of parenteral hyperalimentation. N. Eng. J. Med. 290, 1403 (1974)
Davis A. T., Brunning, R. D., Quie, P. G.: Polymorphonuclear leukocyte myeloperoxidase deficiency in a patient with myelomonocytic leukemia. N. Eng. J. Med. 285, 789 (1971)
DeChatelet, L. R., McPhail, L. C., Mullikin, D., McCall, C. E.: An isotopic assay for NADPH oxidase activity and some characteristics of the enzyme from human polymorphonuclear leukocytes. J. Clin. Invest. 55, 714 (1975).
DeChatelet, L. R., Shirley, P. S., McPhail, L. C.: Normal leukocyte glutathione peroxidase activity in patients with chronic granulomatous disease. J. Pediatr. 89, 598 (1976)
DeMeo, A. N., Anderson, B. R.: Defective chemotaxis associated with a serum inhibitor in cirrhotic patients. N. Engl. J. Med. 286, 735 (1972)
Douglas, S. D., Faulk, W. P.: Immunological aspects of protein calorie malnutrition. In: Recent Advances in Clinical Immunology, Vol. 1, Thompson, R. A., ed., p. 15. New York: Churchill Livingston 1977
Douglas, S. D., Schopfer, K.: Phagocyte function in protein-calorie malnutrition. Clin. Exp. Immunol. 17, 121 (1976)
El-Maalem, H., Fletcher, J.: Impaired neutrophil function and myeloperoxidase deficiency in myeloid metaplasia. Br. J. Haematol. 33, 144, (1976)
El-Maalem, H., Fletcher, J.: Defective neutrophil function in chronic granulocytic leukaemia. Br. J. Haematol. 34, 95 (1976)
Esterson, R. D., Quie, P. G., Hogan, N., Goldberg, N. D.: Cyclic GMP and cell movement. Nature 245, 458 (1973)
Giblett, E. R., Klebanoff, S. J., Pincus, S. H., Swanson, J., Park, B.H., McCullough, J.: Cell phenotypes in chronic granulomatous disease: A potential transfusion hazard. Lancet 1971 I 235
Gray, G. R. Stamatoyannopoulos, G., Naiman, S. C., Kilman, M. R., Klebanoff, S. J., Austin, T., Yoshida, A., Robinson, G. C. F.: Neutrophil dysfunction, chronic granulomatous disease, and nonspherocytic hemolytic anemia caused by complete deficiency of glucose-6-phosphate dehydrogenase. Lancet 1973 II 530
Grignaschi, V. J., Sperperato, A. M., Etcheverry, M. J., Macario, A. J. L.: Un nuevo cuadro citoquimico negatividad espontanea de las reacciones de peroxidasas, oxidasas y lipido en la progenie neutrofilia y en los moncitos de dos hermanos. Rev. Assoc. Med. Argent. 77, 218 (1963)
Gupta, R. C., LaForce, F. M., Mills, D. M.: Polymorphonuclear leukocyte inclusions and impaired bacterial killing in patients with Felty’s syndrome. L. Lab. Clin. Med. 88, 183 (1976)
Henson, P. M., Oades, Z. G.: Enhancement of immunologically induced granule exocytosis from neutrophils by cytochalasin B. J. Cell Biol. 110, 290 (1973)
Higashi, O., Katsuyama, N., Satodate, R.: A case with hematological abnormality characterized by the absence of peroxidase activity in blood polymorphonuclear leukocytes. Tohoku J. Exp. Med. 87, 77 (1965)
Hill, H. R., Quie, P. G.: Raised serum IgE levels and defective neutrophil chemotaxis in three children with eczema and recurrent bacterial infections. Lancet 1975 I, 183
Hill, H. R., Quie, P. G., Ochs, H, H., Clark, R. A., Pabst, H. F., Klebanoff, S. J., Wedgwood, R. J.: Defect in neutrophil granulocyte chemotaxis in Job’s syndrome of recurrent ‘cold’ staphylococcyl abscesses. Lancet 1974 II 617
Hoffstein, S., Zurier, R. B., Weissman, G.: Mechanisms of lysosomal enzyme release from human leukocytes. III. Factors influencing fusion with phagocytic vacuoles and the plasma membrane. Clin. Immunol. Immunopath. 3, 201 (1974)
Hohn, D. C., Lehrer, R. I.: NADPH oxidase deficiency in X-linked chronic granulomatous disease. J. Clin. Invest. 55, 707 (1975)
Holmes, B., Park, B. H., Malawista, S. E., Quie, P. G., Nelson, D. L., Good, R. A.: Chronik granulomatous disease in females: A deficiency of leukocyte glutatione peroxidase. N. Engl. J. Med. 283, 217 (1970)
Johnson, D. E., Griep, J. A., Baehner, R. L.: Histiocytic leukaemia following lifelong infection and thrombocytopenia: Histologic, metabolic, and bacteria studies. J. Pediat, 82, 664 (1973)
Johnston, R. B. Jr., Newman, S. L.: Chronic granulomatous disease. Pediat. Clin North Am. 24, 365 (1977)
Jose, D. G., Shelton, M., Tauro, G. P., Belbin, R. and Hosking, C. S.: Deficiency of immunological and phagocytic function in Aboriginal children with protein calorie malnutrition. Med. J. Aust. 62, 699 (1975)
Klebanoff, S. J.: Myeloperoxidase contribution to the microbicidal activity of intact leukocytes. Science 169, 1095 (1970)
Klebanoff, S. J.: Antimicrobial mechanisms in neutrophilic polymorphonuclear leukocytes. Semin. Hematol. 12, 117 (1975)
Klebanoff, S. J., Pincus, S. H.: Hydrogen peroxide utilization in myeloperoxidase deficient leukocytes: A possible microbicidal control mechanism. J. Clin. Invest. 50, 2226 (1971)
Klebanoff, S. J., White, L. R.: Iodination defect in the leukocytes of a patient with chronic granulomatous disease of childhood. N. Eng. J. Med. 280, 460 (1969)
Kretschmer, R. R., Lopez-Osuna, M., De La Rosa, L.: Leukocyte function in Down’s syndrome: Quantitative NBT reduction and bacterial capacity. Clin. Immunol. Immunopath. 2, 449 (1974)
Lehrer, R. I.: The fungicidal mechanisms of human monocytes. I. Evidence for myeloperoxidaselinked and myeloperoxidase-independent candidacidal mechanisms. J. Clin. Invest. 55, 338 (1975)
Lehrer, R. I., Cline, M. J.: Leukocyte myeloperoxidase deficiency and disseminated candidiasis: The role of myeloperoxidase in resistance to Candida infection. K. Clin. Invest. 48, 1478 (1969)
Lehrer, R. I., Goldberg, L. S., Apple, M. A., Rosenthal, N. P.: Refractory megaloblastic anemia with myeloperoxidase deficient neutrophils. Ann. Intern. Med. 76, 447 (1972)
Lehrer, R. I., Hanifen, J., Cline, M. J.: Defective bactericidal activity in myeloperoxidase-deficient human neutrophils. Nature 223, 78 (1969)
Malawista, S. E., Gee, J. B. L., Bensch, K. G.: Cytochalasin B reversibly inhibits phagocytosis: functional, metabolic and ultrastructural effects in human blood and rabbit alveolar macrophages. Yale K. Biol. Med. 44, 286 (1971)
Malawista, S. E., Gifford, R. H.: Chronic granulomatous disease of childhood (CGD) with leukocyte glutathione peroxidase (LGP) deficiency in a brother and sister: A likely autosomal recessive inheritance. Clin. Res. 23, 416A (1975)
Marsh, W. L., Qyen, R., Nichols, M. E.: Kx antigen, the McCleod phenotype, and chronic granulomatous disease: Further studies. Vox. Sang. 31, 356 (1976)
Matsuda, I., Oka, Y., Taniguchi, N., Furuyama, M., Kodama, S., Arashima, S., Mitsuyamn, T.: Leukocyte glutathione peroxidase deficiency in a male patient with chronic granulomatous disease. J. Pediat. 88, 581 (1976)
McCall, C. E., Caves, J., Cooper, R., DeChatelet, H.: Functional characteristics of human toxic neutrophils. J. Infect. Dis. 124, 68 (1971)
Metchnikoff, E.: Lectures of the Comparative Pathology of Inflammation. New York: Dover Publications 1968
McPhail, L. C., DeChatelet, L. R., Shirley, P. S. Wilfert, C., Johnston, R. B. Jr., McCall, C. E.: Deficiency of NADPH oxidase activity in chronic granulomatous disease. J. Pediatr. 90, 213 (1977)
McPhail, L. C., DeChatelet, L. R., Shirley, P. S. Wilfert, C., Johnston, R. B. Jr., McCall, C. E.: Deficiency of NADPH oxidase activity in chronic granulomatous disease. J. Pediatr. 90, 213 (1977)
Miller, M. E.: Chemotactic function in the human neonate — humoral and cellular aspects. Pediatr. Res. 5, 487 (1971)
Miller, M. E.: Host defense in the human neonate. Pediatr. Clin. North Am. 24, 413 (1977)
Miller, M. E., Dooley, R.: Deficient random mobility, normal chemotaxis and impaired phagocytosis: a new abnormality of neutrophil function. Pediatr. Res. 7, 365A (1973)
Miller, M. E., Norman, M. E., Koblenzer, P. J., Schonauer, T.: A new familial defect of neutrophil movement. J. Lab. Clin. Med. 82, 1 (1973)
Miller, M. E., Oski, F. A., Harris, M. B.: Lazy-leukocyte syndrome: A new disorder of neutrophil function. Lancet 1971 I, 665
Mowat, A., Baum, J.: Chemotaxis of polymorphonuclear leukocytes from patients with diabetes mellitus. N. Engl. J. Med. 284, 621 (1971)
Mowat, A., Baum, J.: Chemotaxis of polymorphonuclear leukocytes from patients with rheumatoid arthritis. J. Clin. Invest. 50, 2541 (1971)
Oliver, J. M., Zurier, R. B.: Correction of characteristic abnormalities of microtubule function and granule morphology in Chediak-Higashi syndrome with cholinergic agonists: Studies in vitro in man and in vivo in the beige mouse. J. Clin. Invest. 57, 1239 (1976)
Oliver, J. M., Zurier, R. B., Berlin, R. D.: Concanavalin B cap formation on polymorphonuclear leukocytes of normal and beige (Chediak-Higashi) mice. Nature 253, 471 (1975)
Olofsson, T., Odenberg, H., Olsson, I.: Granulocyte function in chronic granulocyte leukemia. II. Bactericidal capacity, phagocytic rate, oxygen consumption, and granule protein composition in isolated granulocytes. Blood 48, 581 (1976)
Parmley, R. T., Ogeawa, M., Darby, C. P. Jr., Spicer, S.S.: Congenital neutropenia: Neutrophil proliferation with abnormal maturation. Blood 46, 723 (1975)
Patriarca, P., Cramer, R., Moncalvo, S., Rossi, F., Romeo, D.: Enzymatic basis of metabolic stimulation in leucocytes during phagocytosis: The role of activated NADPH oxidase. Arch. Biochem. Biophys. 145, 255 (1971)
Patriaca, P., Cramer, R., Tedesco, F., Kakinuma, K.: Studies on the mechanism of metabolic stimulation in polymorphonuclear leukocytes during phagocytosis. II. Presence of the NADPH, oxidizing activity in a myeloperoxidase-deficient subject. Biochem. Biophys. Acta 385, 387 (1975)
Pincus, S. H., Thomas, I. T., Clark, R. A., Ochs, H. D.: Defective neutrophil chemotaxis with variant ichthyosis, hyperimmunoglobulinemia E and recurrent infections. J. Pediat. 87, 908 (1975)
Pinkerton, P. H., Robinson, J. B.: Granulocyte function in untreated acute and-chronic granulocytic leukemia. Acta. Haematol. 56, 65 (1976)
Quie, P. G., Cazes, K. L.: Clinical conditions associated with defective polymorphonuclear leukocyte chemotaxis. Am. J. Path. 88, 711 (1977)
Repine, J. E., Clawson, C., Brunning, R. D.: Abnormal pattern of bactericidal activity of neutrophils deficient in granules, myeloperoxidase and alkaline phosphatase. J. Lab. Clin. Med. 88, 788 (1976)
Repine, J. E., Clawson, C. C., Brunning, R. D.: Primary leukocyte alkaline phosphate deficiency in an adult with repeated infections. Br. J. Haematol. 34, 87 (1976)
Root, R. K., Rosenthal, A. S., Balestra, D. J.: Abnormal bactericidal, metabolic and lysosomal functions of Chediak-Higashi syndrome leukocytes. J. Clin. Invest. 51, 649 (1972)
Rosen, H., Klebanoff, S. J.: Chemiluminescence and superoxide production by myeloperoxidase deficient leukocytes. J. Clin. Invest. 58, 50 (1976)
Ruutu, T.: Effect of phenothiazines and related compounds on phagocytosis and bacterial killing by human neutrophilic leukocytes. Ann. Med. Exp. Biol. Fenniae 5t, 24 (1972)
Schopfer, K., Douglas, S. D.: Neutrophil function in children with kwashiorkor. J. Lab. Clin. Med. 88, 450 (1976)
Segal, A. W., Peters, T. J.: Characterization of the enzyme defect in chronic granulomatous disease. Lancet 1976 I, 1363
Singh, H., Boyd, E., Hutton, M. M., Wilkinson, P. C., Pebbles Brown, D. A., Ferguson-Smith, M. A.: Chromosomal mutation in bone marrow as a cause of acquired granulomatous disease and refractory macrocytic anemia. Lancet 1972 I, 873
Synderman, R., Stahl, C.: Defective immune effector function in patients with neoplastic and immune deficiency diseases. In: The Phagocytic Cell and Host Resistance, Bellanti, J., Dayton, D. D., eds., p. 267–281. New York: Raven Press 1975
Solberg, C. O., Schreiner, A., Helium, K. B., Hamre, E.: Neutrophil granulocyte function in the early diagnosis of acute myelomonocytic and myeloblastic leukaemia. Acta Med. Scand. 197, 147 (1975)
Spitznagel, J. K., Cooper, M. R., McCall, C. E., DeChatelet, L. R., Welsh, I. R. H.: Selective deficiency of granules associated with lysozyme and lactoferrin in human polymorphs (PMN) with reduced microbicidal capacity. J. Clin. Invest. 51, 93A (1972)
Steerman, R. L., Synderman, R., Leikin, S. L., Cotten, H. R.: Intrinsic defect of the polymorphonuclear leukocyte resulting in impaired chemotaxis and phagocytosis. Clin. Exp. Immunol. 9, 939 (1971)
Stendahl, O., Lindgren, S.: Function of granulocytes with deficient myeloperoxidase mediated iodination in a patient with generalized pustular psoriasis. Scnad. J. Haematol. 16, 144 (1976)
Stossel, T. P., Root, R. K., Vaughan, M.: Phagocytosis in chronic granulomatous disease and the Chediak-Higashi syndrome. N. Engl. J. Med. 286, 120 (1972)
Strauss, R. G., Bove, K. E., Jones, J. R., Mauer, A. M., Fulginiti, V. A.: An anomaly of neutrophil morphology with impaired function. N. Engl. J. Med. 290, 478 (1974)
Tan, J. S., Strauss, R. G., Akabutu, J., Kauffman, C. A., Mauer, A. M., Phair, J. P.: Persistent neutrophil dysfunction in an adult: Combined defect in chemotaxis, phagocytosis, and intracellular killing. Am. J. Med. 57, 251 (1974)
Undritz, V. E.: Die Alius-Grignaschi anomalie: Der erblich-konstitutionelle peroxydasedefekt der neutrophilen and monozyten. Blut 14, 129 (1966)
Van Epps, D. E., Williams, R. C., Jr.: Suppression of leukocyte chemotaxis by human IgA myeloma components. J. Exp. Med. 144, 1227 (1976)
Van Scoy, R. E., Hill, H. R., Ritts, R. E., Quie, P. G.: Familial neutrophil chemotaxis defect, recurrent bacterial infections, mucocutaneous candidiasis and hyperimmunoglobulinemia E. Ann. Inter. Med. 82, 766 (1975)
Ward, P. A., Schlegel, R. J.: Impaired leukotactic responsiveness in a child with recurrent infections. Lancet 1969 II, 344
Ward, P. A., Berenberg, J. L.: Defective regulation of inflammatory mediators in Hodgkin’s disease. Supernormal levels of chemotactic factor inactivator. N. Engl. J. Med. 290, 76 (1974)
Weissmann, G., Goldstein, I., Hoffstein, S., Tsung, P. K.: Reciprocal effects of cAMP and cGMP on microtubule-dependent release of lysosomal enzymes. Ann. N. Y. Acad. Sci. 253, 750 (1975)
Windhorst, D. B., Katz, E. D.: Normal enzyme activities in chronic granulomatous disease leukocytes. J. Reticuloendothel. Soc. 11, 400 (1972)
Wurster, N., Elsbach, P., Simon, E. J., Peltis, P., LeBow, S.: The effects of morphine analogue levorphanol on leukocytes. J. Clin. Invest. 50, 1091 (1971)
Xanthou, M., Valsssi-Adam, E., Kintzonidou, E., Matsaniotis, N.: Phagocytosis and killing ability of Candila albicans by blood leukocytes of healthy term proterm babies. Arch. Dis. Child. 50, 72 (1971)
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 1979 Springer-Verlag Berlin Heidelberg
About this chapter
Cite this chapter
Quie, P.G., Mills, E.L., McPhail, L.C., Johnston, R.B. (1979). Phagocytic Defects. In: Cooper, M.D., Lawton, A.R., Miescher, P.A., Mueller-Eberhard, H.J. (eds) Immune Deficiency. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-81362-7_6
Download citation
DOI: https://doi.org/10.1007/978-3-642-81362-7_6
Publisher Name: Springer, Berlin, Heidelberg
Print ISBN: 978-3-540-09490-6
Online ISBN: 978-3-642-81362-7
eBook Packages: Springer Book Archive