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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

Oral ulceration in a child with severe congenital neutropenia, who had also suffered from pneumonia and recurrent otitis
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Fig3: Oral ulceration in a child with severe congenital neutropenia, who had also suffered from pneumonia and recurrent otitis

Mentions: Patients with neutropenia frequently present with persistent oral ulcerations, apart from infections with the above-mentioned microorganisms (Fig. 3). Severe neutropenic patients can be treated with G-CSF to reduce the number of infections and increase survival. Unfortunately, long-term treatment with recombinant G-CSF is associated with an increased incidence of leukemia. It has been suggested that the increase in leukemia may be due to the G-CSF treatment. However, it now appears more likely to be due to the increased survival of preleukemic patients [27].Fig. 3


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

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

Oral ulceration in a child with severe congenital neutropenia, who had also suffered from pneumonia and recurrent otitis
© Copyright Policy
Related In: Results  -  Collection

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

Fig3: Oral ulceration in a child with severe congenital neutropenia, who had also suffered from pneumonia and recurrent otitis
Mentions: Patients with neutropenia frequently present with persistent oral ulcerations, apart from infections with the above-mentioned microorganisms (Fig. 3). Severe neutropenic patients can be treated with G-CSF to reduce the number of infections and increase survival. Unfortunately, long-term treatment with recombinant G-CSF is associated with an increased incidence of leukemia. It has been suggested that the increase in leukemia may be due to the G-CSF treatment. However, it now appears more likely to be due to the increased survival of preleukemic patients [27].Fig. 3

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