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Defective bactericidal activity and absence of specific granules in neutrophils from a patient with recurrent bacterial infections

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Abstract

Recent studies have suggested that specific granules and/or their contents may have a role in neutrophil adherence, oxidative metabolism, and other aspects of cell function. In the current report, we studied neutrophil function in a 13-year-old female with recurrent pyogenic infections and absent specific granules previously documented by electron microscopy. Levels of cobalamin (vitamin B12)-binding protein and lactoferrin were markedly decreased in this patient's neutrophils. Bactericidal activity againstEscherichia coli was decreased at 60 and 120 min (percentage organisms killed: patient neutrophils, 48 and 33%; control neutrophils, 90 and 99%, respectively). Defective killing ofStaphylococcus aureus was also documented. Degranulation and adherence were normal. Levels of lactoferrin and cobalamin-binding protein were decreased in plasma but normal in saliva, indicating that the defect was specific for hematopoietic tissue. Superoxide anion production was normal in the patient, while hydroxyl radical generation was decreased in response to opsonized zymosan. The data support the concept that specific granules and their contents are important for oxidative metabolism and other neutrophil functions.

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Authors and Affiliations

  1. Section of Hematology/Oncology, Department of Pediatrics, University of Colorado School of Medicine, and the Belle Bonfils Memorial Blood Center, 80262, Denver, Colorado

    Daniel R. Ambruso

  2. First Division, Department of Internal Medicine, Kyoto University, Kyoto, Japan

    Masataka Sasada, Hideki Nishiyama & Akemi Kubo

  3. Department of Pediatrics, Sinsu University School of Medicine, Matsumoto, Japan

    Atsushi Komiyama

  4. Division of Hematology, Department of Internal Medicine, University of Colorado School of Medicine, 80262, Denver, Colorado

    Robert H. Allen

Authors
  1. Daniel R. Ambruso
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  2. Masataka Sasada
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  3. Hideki Nishiyama
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  4. Akemi Kubo
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  5. Atsushi Komiyama
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  6. Robert H. Allen
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Ambruso, D.R., Sasada, M., Nishiyama, H. et al. Defective bactericidal activity and absence of specific granules in neutrophils from a patient with recurrent bacterial infections. J Clin Immunol 4, 23–30 (1984). https://doi.org/10.1007/BF00915283

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  • DOI: https://doi.org/10.1007/BF00915283

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