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Chapter 77 : Hereditary and Acquired Complement Deficiencies

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Hereditary and Acquired Complement Deficiencies, Page 1 of 2

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Abstract:

Complement deficiencies comprise a small but important category of primary immune deficiency diseases. Complement deficiency states can also be acquired from activation or inhibition of the complement system, temporarily depleting components faster than they can be replaced or causing changes in control of the system. Complement plays an important role in the host's response to infection by directly killing bacteria, neutralizing viruses, or coating microbial surfaces with complement fragments that enhance uptake and killing by phagocytes, a process called opsonization that ensures that objects identified as foreign by complement are dealt with quickly and efficiently. Complement serves as a link between many of the activities of acquired immunity, with ties to diverse cell signal responses and other defense mechanisms, as an ever-increasing number of related conditions are identified (1).

Citation: Giclas P. 2016. Hereditary and Acquired Complement Deficiencies, p 749-765. 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.ch77
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FIGURE 1

Complement was one of the earliest members of the innate immune system to evolve, and, as such, different forms of pattern recognition allowed it to become active against a variety of organisms and substances without the need for prior exposure. The three boxes to the left (classical pathway [CP], blue; lectin pathway [LP], orange; alternative pathway [AP], red) indicate examples of one of the triggers for activation of each pathway. As drawn, the C3 molecule is the central target for all three pathways. Activation of the CP by immune complexes, or the LP by a recognized target pattern, results in cleavage of C3, C4, and C2. Clearance of the complex or LP target from the circulation occurs through interaction of the phagocytes in the liver and spleen with the C4b and C3b bound to the complex. The alternative pathway is different. In the AP, the unique protein properdin allows the formation of an amplification process (shown as circle of arrows) in which a very large amount of C3 but not C4 becomes rapidly cleaved. The resulting C3b from this process becomes covalently bound to the surface of nearby proteins, microbes, and cells and flags them as targets for destruction by the immune system. The fragments of C3 and C4 form the major opsonins that are used by cells such as phagocytes and lymphocytes to clear the C3 and C4 fragments. Note that complement fragments can be useful in the laboratory to identify the pathway involved in a reaction. Note also that a large decrease in serum C3 but not C4 is indicative of AP activation. The majority of the physiological effects of complement activation are due to the inflammatory properties of the fragments. These include opsonization, chemotaxis, mediator release, priming of phagocytes and lymphocytes, apoptosis, and direct killing of microbes by lysis.

Citation: Giclas P. 2016. Hereditary and Acquired Complement Deficiencies, p 749-765. 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.ch77
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Tables

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TABLE 1

Testing for complement deficiency or dysfunction

Citation: Giclas P. 2016. Hereditary and Acquired Complement Deficiencies, p 749-765. 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.ch77

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