Severe congenital neutropenia: Clinical effects and neutrophil function during treatment with granulocyte colony-stimulating factor

Brent Weston, Robert F. Todd, Richard Axtell, Kenneth Balazovich, Jeffrey Stewart, Betty J. Locey, Laura Mayo-Bond, Paul Loos, Raymond Hutchinson, Laurence A. Boxer

Research output: Contribution to journalArticle

34 Scopus citations

Abstract

We studied neutrophil function and clinical responses in seven patients with severe congenital neutropenia (SCN) after they received treatment with recombinant human granulocyte colony stimulating factor (rhG-CSF). Two subpopulations of patients with SCN were defined by their pattern of absolute neutrophil response, superoxide production, and cytochrome b559 levels. One group had an oscillating absolute neutrophil count and reduced ability to produce superoxide and cytochrome b559 (n = 4), and the second group had a relatively constant absolute neutrophil count response with normal superoxide and cytochrome levels (n = 3). Neutrophils from both groups had decreased surface expression of FcRIII and abnormal upregulation of the C3bi receptor (CR3). All patient neutrophils, however, had normal contents of the primary granule constituent, β-glucuronidase, and the specific granule constituent, vitamin B 12 binding protein. The clinical response to rhG-CSF was evident by marked improvement in the degree of periodontitis and reduction in the number of oral ulcers in both groups of patients. Although neutrophil function is not completely normal in patients with SCN, it is likely that enough redundancy exists in neutrophil bactericidal capacity to promote normal host response to inflammation.

Original languageEnglish (US)
Pages (from-to)282-290
Number of pages9
JournalThe Journal of Laboratory and Clinical Medicine
Volume117
Issue number4
StatePublished - Apr 1991

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

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    Weston, B., Todd, R. F., Axtell, R., Balazovich, K., Stewart, J., Locey, B. J., Mayo-Bond, L., Loos, P., Hutchinson, R., & Boxer, L. A. (1991). Severe congenital neutropenia: Clinical effects and neutrophil function during treatment with granulocyte colony-stimulating factor. The Journal of Laboratory and Clinical Medicine, 117(4), 282-290.