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Chapter 78 : Neutropenia and Neutrophil Defects

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Neutropenia and Neutrophil Defects, Page 1 of 2

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Abstract:

Concern about the neutrophil status of a patient is usually raised on the basis of the frequency, severity, and distribution of a specific infectious agent(s) involved in one or more episodes that are, or are thought to be, infectious. The clinical presentations of patients with neutrophil disorders usually share a few common features: gingivitis, periodontal disease, and oral ulceration. Cutaneous infections with are often recurrent and can be severe. In neutrophil disorders characterized by inadequate inflammation (neutropenia, leukocyte adhesion deficiency [LAD], Chédiak-Higashi syndrome, and specific granule deficiency), infections can extend locally and subcutaneously with little reaction until marked destruction has taken place. Clinically relevant neutrophil abnormalities fall into several broad categories: neutropenia, abnormalities of neutrophil adherence and locomotion, abnormalities of neutrophil granule formation or content, and abnormalities of killing. With the widespread use of therapies which modulate the immune system either by design (e.g., steroids and monoclonal antibodies) or incidentally (e.g., cytotoxic chemotherapy), the most common causes of immunodeficiency are iatrogenic. The recognition, characterization, identification, and cloning of disease-related genes and, in some cases, genetic correction of immune defects are progressing rapidly. As specific and genetic treatments become available, making the correct diagnosis early takes on greater therapeutic importance (1).

Citation: Holland S. 2016. Neutropenia and Neutrophil Defects, p 766-774. In Detrick B, Schmitz J, Hamilton R (ed), Manual of Molecular and Clinical Laboratory Immunology, Eighth Edition. ASM Press, Washington, DC. doi: 10.1128/9781555818722.ch78
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References

/content/book/10.1128/9781555818722.ch78
1. Rosenzweig SD, Holland SM. 2011. Recent insights into the pathobiology of innate immune deficiencies. Curr Allergy Asthma Rep 11:369377.[CrossRef].[PubMed]
2. Newburger PE, Dale DC. 2013. Evaluation and management of patients with isolated neutropenia. Semin Hematol 50:198206.[CrossRef].[PubMed]
3. Akhtari M, Curtis B, Waller EK. 2009. Autoimmune neutropenia in adults. Autoimmun Rev 9:6266.[CrossRef].[PubMed]
4. Leiding JW, Holland SM,. 2013. Chronic granulomatous disease, p 19932013. In Pagon RA, Adam MP, Bird TD, Dolan CR, Fong CT, Stephens K (ed), GeneReviews. University of Washington, Seattle, Seattle, WA.
5. Elloumi HZ, Holland SM. 2007. Diagnostic assays for chronic granulomatous disease and other neutrophil disorders. Methods Mol Biol 412:505523.[CrossRef].[PubMed]
6. van de Vijver E, van den Berg TK, Kuijpers TW. 2013. Leukocyte adhesion deficiencies. Hematol Oncol Clin North Am 27:101116, viii.[CrossRef].[PubMed]
7. Mauch L, Lun A, O'Gorman MR, Harris JS, Schulze I, Zychlinsky A, Fuchs T, Oelschlägel U, Brenner S, Kutter D, Rösen-Wolff A, Roesler J. 2007. Chronic granulomatous disease (CGD) and complete myeloperoxidase deficiency both yield strongly reduced dihydrorhodamine 123 test signals but can be easily discerned in routine testing for CGD. Clin Chem 53:890896.[CrossRef].[PubMed]
8. Kuhns DB, Alvord WG, Heller T, Feld JJ, Pike KM, Marciano BE, Uzel G, DeRavin SS, Priel DA, Soule BP, Zarember KA, Malech HL, Holland SM, Gallin JI. 2010. Residual NADPH oxidase and survival in chronic granulomatous disease. N Engl J Med 363:26002610.[CrossRef].[PubMed]
9. Segal BH, Leto TL, Gallin JI, Malech HL, Holland SM. 2000. Genetic, biochemical, and clinical features of chronic granulomatous disease. Medicine (Baltimore) 79:170200.[PubMed].[CrossRef]
10. Metcalf JA, Gallin JI, Nauseef WM, Root RK. 1986. Laboratory Manual of Neutrophil Function. Raven Press, New York, NY.

Tables

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TABLE 1

Causes of neutropenia

Citation: Holland S. 2016. Neutropenia and Neutrophil Defects, p 766-774. In Detrick B, Schmitz J, Hamilton R (ed), Manual of Molecular and Clinical Laboratory Immunology, Eighth Edition. ASM Press, Washington, DC. doi: 10.1128/9781555818722.ch78
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TABLE 2

Neutrophil defects

Citation: Holland S. 2016. Neutropenia and Neutrophil Defects, p 766-774. In Detrick B, Schmitz J, Hamilton R (ed), Manual of Molecular and Clinical Laboratory Immunology, Eighth Edition. ASM Press, Washington, DC. doi: 10.1128/9781555818722.ch78
Generic image for table
Untitled

Citation: Holland S. 2016. Neutropenia and Neutrophil Defects, p 766-774. In Detrick B, Schmitz J, Hamilton R (ed), Manual of Molecular and Clinical Laboratory Immunology, Eighth Edition. ASM Press, Washington, DC. doi: 10.1128/9781555818722.ch78
Generic image for table
Untitled

Citation: Holland S. 2016. Neutropenia and Neutrophil Defects, p 766-774. In Detrick B, Schmitz J, Hamilton R (ed), Manual of Molecular and Clinical Laboratory Immunology, Eighth Edition. ASM Press, Washington, DC. doi: 10.1128/9781555818722.ch78
Generic image for table
Untitled

Citation: Holland S. 2016. Neutropenia and Neutrophil Defects, p 766-774. In Detrick B, Schmitz J, Hamilton R (ed), Manual of Molecular and Clinical Laboratory Immunology, Eighth Edition. ASM Press, Washington, DC. doi: 10.1128/9781555818722.ch78
Generic image for table
Untitled

Citation: Holland S. 2016. Neutropenia and Neutrophil Defects, p 766-774. In Detrick B, Schmitz J, Hamilton R (ed), Manual of Molecular and Clinical Laboratory Immunology, Eighth Edition. ASM Press, Washington, DC. doi: 10.1128/9781555818722.ch78
Generic image for table
Untitled

Citation: Holland S. 2016. Neutropenia and Neutrophil Defects, p 766-774. In Detrick B, Schmitz J, Hamilton R (ed), Manual of Molecular and Clinical Laboratory Immunology, Eighth Edition. ASM Press, Washington, DC. doi: 10.1128/9781555818722.ch78
Generic image for table
Untitled

Citation: Holland S. 2016. Neutropenia and Neutrophil Defects, p 766-774. In Detrick B, Schmitz J, Hamilton R (ed), Manual of Molecular and Clinical Laboratory Immunology, Eighth Edition. ASM Press, Washington, DC. doi: 10.1128/9781555818722.ch78
Generic image for table
Untitled

Citation: Holland S. 2016. Neutropenia and Neutrophil Defects, p 766-774. In Detrick B, Schmitz J, Hamilton R (ed), Manual of Molecular and Clinical Laboratory Immunology, Eighth Edition. ASM Press, Washington, DC. doi: 10.1128/9781555818722.ch78
Generic image for table
Untitled

Citation: Holland S. 2016. Neutropenia and Neutrophil Defects, p 766-774. In Detrick B, Schmitz J, Hamilton R (ed), Manual of Molecular and Clinical Laboratory Immunology, Eighth Edition. ASM Press, Washington, DC. doi: 10.1128/9781555818722.ch78

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