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Chapter 98 : The Primary Immunodeficiency Diseases

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The Primary Immunodeficiency Diseases, Page 1 of 2

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Abstract:

Patients with primary immunodeficiency diseases most often are recognized because of their increased susceptibility to infection (chronic or recurrent infections without other explanation, infection with an organism of low virulence, or infection of unusual severity). The type of pathogen and the location of the infection may give valuable insight into the nature of the immunologic defect. Individuals with complement deficiencies most often present with bacteremia, septic arthritis, and meningitis, caused by encapsulated bacteria. Phagocytic disorders are characterized by bacterial and fungal infections of the skin and abscesses in the reticuloendothelial system (lymph nodes, spleen, and liver). Autoimmune and inflammatory diseases are more commonly seen in particular primary immunodeficiency diseases, most notably common variable immunodeficiency, selective immunoglobulin A (IgA) deficiency, chronic mucocutaneous candidiasis, and deficiencies of early components of the classical complement pathway (C1 to C4). Although immune system dysfunction can be suspected by the clinician after careful review of the history and physical exam, specific diagnoses are rarely evident without the use of the laboratory. However, the types of infections and other symptoms should help to focus the laboratory workup on specific compartments of the immune system. Finally, whenever primary immunodeficiency is suspected, consideration must also be given to secondary causes of immunodeficiency, including human immunodeficiency virus infection, therapy with anti-inflammatory medications (e.g., corticosteroids), and other underlying illnesses (e.g., lymphoreticular neoplasms and viral infections, such as infectious mononucleosis).

Citation: Lederman H. 2006. The Primary Immunodeficiency Diseases, p 893-894. In Detrick B, Hamilton R, Folds J (ed), Manual of Molecular and Clinical Laboratory Immunology, 7th Edition. ASM Press, Washington, DC. doi: 10.1128/9781555815905.ch98

Key Concept Ranking

Systemic Lupus Erythematosus
0.51751256
Immune Systems
0.5094729
Common Variable Immunodeficiency
0.45336896
Severe Combined Immunodeficiency
0.45336896
0.51751256
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References

/content/book/10.1128/9781555815905.ch98
1. Bonilla, F. A., and , R. S. Geha. 2003. Primary immunodeficiency diseases. J. Allergy Clin. Immunol. 111:S571S581.
2. Chapel, H.,, R. Geha, and F. Rosin for the IUIS PID Classification Committee. 2003. Primary immunodeficiency diseases: an update. Clin. Exp. Immunol. 132:915.
3. Folds, J. D., and , J. L. Schmitz. 2003. Clinical and laboratory assessment of immunity. J. Allergy Clin. Immunol. 111:S702S711.
4. Stiehm, E. R.,, H. D. Ochs, and , J. A. Winkelstein. 2004. Immunodeficiency disorders: general considerations, p. 289355. In E. R. Stiehm,, H. D. Ochs, and , J. A. Winkelstein (ed.), Immunologic Disorders in Infants and Children, 5th ed. Elsevier Saunders, Philadelphia, Pa.

Tables

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TABLE 1

Examples of immunodeficiency syndromes

Citation: Lederman H. 2006. The Primary Immunodeficiency Diseases, p 893-894. In Detrick B, Hamilton R, Folds J (ed), Manual of Molecular and Clinical Laboratory Immunology, 7th Edition. ASM Press, Washington, DC. doi: 10.1128/9781555815905.ch98
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TABLE 2

Patterns of illness associated with primary immunodeficiency

Citation: Lederman H. 2006. The Primary Immunodeficiency Diseases, p 893-894. In Detrick B, Hamilton R, Folds J (ed), Manual of Molecular and Clinical Laboratory Immunology, 7th Edition. ASM Press, Washington, DC. doi: 10.1128/9781555815905.ch98
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TABLE 3

Screening tests for primary immunodeficiency

Citation: Lederman H. 2006. The Primary Immunodeficiency Diseases, p 893-894. In Detrick B, Hamilton R, Folds J (ed), Manual of Molecular and Clinical Laboratory Immunology, 7th Edition. ASM Press, Washington, DC. doi: 10.1128/9781555815905.ch98

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