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Inflammatory consequences of inherited disorders affecting neutrophil function

Mary C. Dinauer

Abstract

Primary immunodeficiencies affecting the function of neutrophils and other phagocytic leukocytes are notable for an increased susceptibility to bacterial and fungal infections as a result of impaired leukocyte recruitment, ingestion, and/ or killing of microbes. The underlying molecular defects can also impact other innate immune responses to infectious and inflammatory stimuli, leading to inflammatory and autoimmune complications that are not always directly related to infection. This review will provide an update on congenital disorders affecting neutrophil function where a combination of host defense and inflammatory complications are prominent, including NADPH oxidase defects in chronic granulomatous disease and β2 integrin defects in leukocyte adhesion deficiency.

  • Submitted November 29, 2018.
  • Accepted January 13, 2019.