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A neutrophil lysozyme leak in patients with familial Mediterranean fever

M Bar-Eli, M C Territo, R S Peters

    American Journal of Hematology
    |December 1, 1981
    PubMed

    Insights

    Familial Mediterranean Fever (FMF) neutrophils release more lysozyme when exposed to heat. This heightened lysozyme release, specific to FMF neutrophils, may play a role in FMF pathogenesis.

    Area of Science:

    • Immunology
    • Genetics
    • Biochemistry

    Background:

    • Familial Mediterranean Fever (FMF) is a genetic autoinflammatory disorder.
    • Neutrophils are key immune cells involved in inflammatory responses.
    • Abnormalities in neutrophil function may contribute to FMF pathogenesis.

    Purpose of the Study:

    • To investigate the release of specific enzymes from neutrophils in patients with FMF.
    • To determine the effect of heat stress on neutrophil enzyme release in FMF.
    • To explore the potential role of neutrophil lysozyme in FMF.

    Main Methods:

    • Peripheral blood polymorphonuclear cells (PMNs) were isolated from FMF patients and healthy controls.
    • PMNs were exposed to elevated temperatures (42°C and 46°C).
    • The release of lysozyme, acid phosphatase, beta-glucuronidase, and lactoferrin was measured.

    Main Results:

    • FMF neutrophils showed significantly increased lysozyme release at high temperatures compared to controls.
    • No differences in the release of acid phosphatase, beta-glucuronidase, or lactoferrin were observed.
    • Colchicine treatment did not affect lysozyme release from FMF neutrophils.
    • Increased lysozyme release was specific to FMF neutrophils and not observed in other inflammatory conditions or monocytes.

    Conclusions:

    • Elevated temperature specifically enhances lysozyme release from FMF neutrophils.
    • This heightened lysozyme release may be a significant factor in the pathogenesis of Familial Mediterranean Fever.
    • Further research is warranted to elucidate the precise mechanisms and implications of this finding.

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