Congenital and Acquired Lactoferrin Deficiencies in Neutrophils

  • Jean-Louis Vildé
  • Janine Breton-Gorius
  • Jacques Hakim
  • Diego Buriot
  • Claude Griscelli
  • R. A. Harkness
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 141)


Human polymorphonuclear leukocytes (PMN) contain two main types of granules: the primary or azurophil granules and the secondary or specific granules 1. Each one can be recognized by specific markers of their content : myeloperoxidase (MPO) for the former and lactoferrin (LF) for the latter. The presence of LF, an iron-binding protein 2, in human PMN was demonstrated by Masson3 in 1969 and its localization in specific granules by Baggiolini 4 in 1970 and Mason 5. Until today, four cases of absence of specific granules in human PMN have been reported 6–9. Our purpose is to describe the morphologic, immunocytochemical and functional features of PMN from a child which were shown to lack specific granules and LF, and from some patients who had the same but acquired defects, and to discuss the role of specific granules and LF in the resistance to infection.


Serratia Marcescens Azurophil Granule Human Lactoferrin Human Polymorphonuclear Leukocyte Recurrent Bacterial Infection 
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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  1. 1.
    D.F. Bainton, J.L. Ullyot, and M.G. Farqhar, The development of neutrophilic polymorphonuclear leukocytes in human bone marrow. Origin and content of azurophil and specific granules, J. Exp. Med. 134 907 (1971).PubMedCrossRefGoogle Scholar
  2. 2.
    S.J. Klebanoff and R.A. Clark, “The neutrophils: function and clinical disorders” North Holland Publishing Company, Amsterdam (1978).Google Scholar
  3. 3.
    P.L. Masson, J.F. Heremans, and E.Schonne, Lactoferrin, an iron-binding protein in neutrophilic leukocytes, J. Exp. Med. 130: 643 (1969).PubMedCrossRefGoogle Scholar
  4. 4.
    M. Baggiolíni, C. de Duve, P.L. Masson, and J.F. Heremans, Association of lactoferrin with specific granules in rabbit heterophil leukocytes, J. Exp. Med. 131: 559 (1970).PubMedCrossRefGoogle Scholar
  5. 5.
    D.Y. Mason, C. Farrell, and C.R. Taylor, The detection of intracellular antigens in human leukocytes by immunoperoxidase staining, Br. J. Haematol. 31: 361 (1975).PubMedCrossRefGoogle Scholar
  6. 6.
    J.K. Spitznagel, M.R. Cooper, A.E. Mc Call, L.R. Dechatelet, and I.R.H. Welsh, Selective deficiency of granules associated with lysozyme and lactoferrin in human polymorphs (PMN) with reduced microbicidal capacity, J. Clin. Invest. 51: 93 a (1972).Google Scholar
  7. 7.
    R.G. Strauss, K.E. Boue, J.F. Jones, A.M.• Mauer, and V.A. Fulgíniti, An anomaly of neutrophil morphology with impaired function, N. Engl. J. Med. 290: 478 (1974).PubMedCrossRefGoogle Scholar
  8. 8.
    R.T. Parmley, M. Ogawa, C.P. Darby Jr, and S.S. Spicer, Congenital neutropenia: neutrophil proliferation with abnormal maturation, Blood 46: 723 (1975)PubMedGoogle Scholar
  9. 9.
    A. Komiyana, H. Morosawa, T. Nakahata, Y. Miyagawa, and T. Akabane, Abnormal neutrophil maturation in a neutrophil defect with morphologic abnormality and impaired function, J. Pediatr. 94: 19 (1979).CrossRefGoogle Scholar
  10. 10.
    J.Breton-Gorius, D.Y. Mason, D.Buriot, J-L. Vildé, and C. Griscellí, Lactoferrin deficiency as a consequence of a lack of specific granules in neutrophils from a patient with recurrent infections, Am. J. Path. 99: 413 (1980).PubMedGoogle Scholar
  11. 11.
    J. Breton-Gorius, D. Houssay, and B. Dreyfus, Partial myeloperoxidase deficiency in a case of preleukemía: I Studies of fine structure and peroxídase synthesis of promyelocytes, Brit. J. Haematol. 30: 273 (1975).CrossRefGoogle Scholar
  12. 12.
    D. Buriot, J. Breton-Gorius, C. Griscelli, I. Olsson, and J. Hakim, Specific granules and lactoferrin deficiencies of neutrophils in a child with repeated infections (submitted for publication).Google Scholar
  13. 13.
    J. Breton-Gorius, D. Houssay, J-L. Vildé, and B. Dreyfus, Partial myeloperoxidase deficiency in a case of preleukemia. II Defects of degranulation and abnormal bactericidal activity of blood neutrophils, Brit. J. Haematol. 30: 279 (1975).CrossRefGoogle Scholar
  14. 14.
    D.R. Ambruso, and R.B. Johnston Jr, Lactoferrin enhances hydroxyl radical (.OH) production by a particulate fraction (PF) of neutrophils, Clin. Res. 28: 490 A (1980).Google Scholar
  15. 15.
    D.F.Bainton, Sequential degranulation of the two types of polymorphonuclear leukocyte granules during phagocytosis of microorganisms, J. Cell. Biol. 58: 249 (1973)PubMedCrossRefGoogle Scholar
  16. 16.
    J.G.White, and R.D. Estensen, Selective labilization of specific granules in polymorphonuclear leukocytes by phorbol myristate acetate, Am. J. Path. 75: 45 (1974).PubMedGoogle Scholar
  17. 17.
    R.R. Arnold, M.F. Cole, and J.R. Mc Ghee, A bactericidal effect for human lactoferrin, Science 197: 263 (1977).PubMedCrossRefGoogle Scholar
  18. 18.
    J.J. Bullen, and J.A. Armstrong, The role of lactoferrin in the bactericidal function of polymorphonuclear leukocytes, Immunology 36: 781 (1979).PubMedGoogle Scholar
  19. 19.
    M.S. Lefell, and J.K. Spitznagel, Fate of human lactoferrin and myeloperoxidase in phagocytosing human neutrophile: effects of immunoglobulin G subclasses and immune complexes coated on latex beads, Infect. Immun. 12: 813 (1975).Google Scholar

Copyright information

© Plenum Press, New York 1982

Authors and Affiliations

  • Jean-Louis Vildé
    • 1
  • Janine Breton-Gorius
    • 2
  • Jacques Hakim
    • 3
  • Diego Buriot
    • 4
  • Claude Griscelli
    • 4
  • R. A. Harkness
  1. 1.Hopital Claude BernardParisF.
  2. 2.INSERM U91 Hopital Henri MondorCréteilF.
  3. 3.Hopital BichatParisF.
  4. 4.Hopital des Enfants-MaladesParisF.

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