Analysis of the Complement System in the Clinical Immunology Laboratory

被引：70
|
作者：
Ling, Morris [1 ,2 ,3 ,4 ]
Murali, Mandakolathur [1 ,3 ,4 ]
机构：
[1] Massachusetts Gen Hosp, Dept Med, Div Rheumatol Allergy & Immunol, 55 Fruit St,Cox 201, Boston, MA 02114 USA
[2] Massachusetts Gen Hosp, Ctr Immunol & Inflammatory Dis, 55 Fruit St,Cox 201, Boston, MA 02114 USA
[3] Massachusetts Gen Hosp, Dept Pathol, 55 Fruit St,Cox 201, Boston, MA 02114 USA
[4] Harvard Med Sch, Dept Med, 55 Fruit St,Cox 201, Boston, MA 02114 USA
来源：
CLINICS IN LABORATORY MEDICINE | 2019年 / 39卷 / 04期
关键词：
Complement; CH50; C3; C4; Classical pathway; Lectin pathway; Alternative pathway; Membrane attack complex; HEMOLYTIC-UREMIC-SYNDROME; PAROXYSMAL-NOCTURNAL HEMOGLOBINURIA; PIG-A GENE; FACTOR-H; HEREDITARY ANGIOEDEMA; MUTATIONS; PROTEIN; PREDISPOSE; RECEPTORS; MCP;
D O I：
10.1016/j.cll.2019.07.006
中图分类号：
R446 [实验室诊断]; R-33 [实验医学、医学实验];
学科分类号：
1001 ;
摘要：
The complement system is a critical component of both the innate and adaptive immune systems that augments the function of antibodies and phagocytes. Antigen-antibody immune complexes, lectin binding, and accelerated C3 tick-over can activate this well-coordinated and carefully regulated process. The importance of this system is highlighted by the disorders that arise when complement components or regulators are deficient or dysregulated. This article describes the pathways involved in complement activation and function, the regulation of these various pathways, and the interpretation of laboratory testing performed for the diagnosis of diseases of complement deficiency, exuberant complement activation, and complement dysregulation.
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页码：579 / +
页数：13
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