Inflammatory consequences of inherited disorders affecting neutrophil function

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 a...

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Bibliographic Details
Published in:Blood 2019-05, Vol.133 (20), p.2130-2139
Main Author: Dinauer, Mary C.
Format: Article
Language:English
Subjects:
Animals
Autoimmune Diseases - etiology
Autoimmune Diseases - genetics
Autoimmune Diseases - immunology
Autoimmune Diseases - pathology
Genetic Variation
Granulomatous Disease, Chronic - complications
Granulomatous Disease, Chronic - genetics
Granulomatous Disease, Chronic - immunology
Granulomatous Disease, Chronic - pathology
Human Neutrophils
Humans
Immunity, Innate
Inflammation - etiology
Inflammation - genetics
Inflammation - immunology
Inflammation - pathology
NADPH Oxidases - genetics
NADPH Oxidases - immunology
Neutrophils - immunology
Neutrophils - pathology
Review Series
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Summary: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 in which a combination of host defense and inflammatory complications are prominent, including nicotinamide dinucleotide phosphate oxidase defects in chronic granulomatous disease and β2 integrin defects in leukocyte adhesion deficiency.
ISSN:0006-4971
1528-0020
DOI:10.1182/blood-2018-11-844563