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Educational paper: Defects in number and function of neutrophilic granulocytes causing primary immunodeficiency.

van den Berg JM, Kuijpers TW - Eur. J. Pediatr. (2011)

Bottom Line: The neutrophilic granulocyte (neutrophil) is the most important cellular component of the innate immune system.A total absence of neutrophils or a significant decrease in their number leads to severe immunodeficiency.In order to be neutralized, this pathogen has to be recognized, phagocytosed, and destroyed by lytic enzymes contained in the neutrophil's granules and reactive oxygen species formed by the enzyme complex NADPH oxidase.

View Article: PubMed Central - PubMed

Affiliation: Dept of Pediatric Hematology, Immunology and Infectious Diseases, Emma Children's Hospital, Academic Medical Centre, University of Amsterdam, Room H7-214, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands. j.m.vandenberg@amc.nl

ABSTRACT
The neutrophilic granulocyte (neutrophil) is the most important cellular component of the innate immune system. A total absence of neutrophils or a significant decrease in their number leads to severe immunodeficiency. A mature neutrophil, released from the bone marrow, should be able to migrate from the blood towards the tissues, following a chemotactic gradient to a pathogen. In order to be neutralized, this pathogen has to be recognized, phagocytosed, and destroyed by lytic enzymes contained in the neutrophil's granules and reactive oxygen species formed by the enzyme complex NADPH oxidase. Rare genetic defects leading to the loss of each one of these biological properties of the neutrophil have been described and are associated with immunodeficiency. This review provides a summary of the normal development and biological functions of neutrophils and describes the diseases caused by defects in neutrophil number and function.

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Related in: MedlinePlus

Two granulomatous lesions in the thymus of an X-linked CGD patient
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Fig6: Two granulomatous lesions in the thymus of an X-linked CGD patient

Mentions: Chronic granulomatous disease (CGD) is the most frequent disorder of neutrophil function. In CGD, components of an enzyme complex named NADPH oxidase are absent, which leads to an inability to kill phagocytosed microorganisms. Apart from fulminant infections by bacteria and fungi, this also leads to chronic, low-grade inflammatory reactions characterized by granuloma formation. In the gut, this process is indistinguishable from Crohn's disease, in the lung CGD that can mimic sarcoidosis [25] (Fig. 6).Fig. 6


Educational paper: Defects in number and function of neutrophilic granulocytes causing primary immunodeficiency.

van den Berg JM, Kuijpers TW - Eur. J. Pediatr. (2011)

Two granulomatous lesions in the thymus of an X-linked CGD patient
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC3197933&req=5

Fig6: Two granulomatous lesions in the thymus of an X-linked CGD patient
Mentions: Chronic granulomatous disease (CGD) is the most frequent disorder of neutrophil function. In CGD, components of an enzyme complex named NADPH oxidase are absent, which leads to an inability to kill phagocytosed microorganisms. Apart from fulminant infections by bacteria and fungi, this also leads to chronic, low-grade inflammatory reactions characterized by granuloma formation. In the gut, this process is indistinguishable from Crohn's disease, in the lung CGD that can mimic sarcoidosis [25] (Fig. 6).Fig. 6

Bottom Line: The neutrophilic granulocyte (neutrophil) is the most important cellular component of the innate immune system.A total absence of neutrophils or a significant decrease in their number leads to severe immunodeficiency.In order to be neutralized, this pathogen has to be recognized, phagocytosed, and destroyed by lytic enzymes contained in the neutrophil's granules and reactive oxygen species formed by the enzyme complex NADPH oxidase.

View Article: PubMed Central - PubMed

Affiliation: Dept of Pediatric Hematology, Immunology and Infectious Diseases, Emma Children's Hospital, Academic Medical Centre, University of Amsterdam, Room H7-214, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands. j.m.vandenberg@amc.nl

ABSTRACT
The neutrophilic granulocyte (neutrophil) is the most important cellular component of the innate immune system. A total absence of neutrophils or a significant decrease in their number leads to severe immunodeficiency. A mature neutrophil, released from the bone marrow, should be able to migrate from the blood towards the tissues, following a chemotactic gradient to a pathogen. In order to be neutralized, this pathogen has to be recognized, phagocytosed, and destroyed by lytic enzymes contained in the neutrophil's granules and reactive oxygen species formed by the enzyme complex NADPH oxidase. Rare genetic defects leading to the loss of each one of these biological properties of the neutrophil have been described and are associated with immunodeficiency. This review provides a summary of the normal development and biological functions of neutrophils and describes the diseases caused by defects in neutrophil number and function.

Show MeSH
Related in: MedlinePlus