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Detection of individuals with an ongoing immune process
First-tier screening test for congenital complement deficiencies
1. Immediately after specimen collection, place the tube on wet ice.
2. Centrifuge and separate serum from clot; transferring the serum into a plastic vial.
3. Immediately freeze specimen.
Complement proteins are components of the innate immune system. There are 3 pathways to complement activation: (1) the classic pathway, (2) the alternative (or properdin) pathway, and (3) the lectin activation (or mannan-binding protein) pathway. The classic pathway of the complement system is composed of a series of proteins that are activated in response to the presence of immune complexes. The activation process results in the generation of peptides that are chemotactic for neutrophils and that bind to immune complexes and complement receptors. The end result of the complement activation cascade is the formation of the lytic membrane attack complex (MAC).
The absence of early components (C1, C2, C3, C4) of the complement cascade results in the inability of immune complexes to activate the cascade. Patients with deficiencies of the early complement proteins are unable to generate the peptides that are necessary to clear immune complexes and to attract neutrophils or to generate lytic activity. These patients have increased susceptibility to infections with encapsulated microorganisms. They may also have symptoms that suggest autoimmune disease, and complement deficiency may be an etiologic factor in the development of autoimmune disease.
Patients with deficiencies of the late complement proteins (C5, C6, C7, C8, and C9) are unable to form the MAC, and may have increased susceptibility to neisserial infections.
Undetectable complement levels are found in patients with specific component deficiencies. Decreased complement levels are found in infectious and autoimmune diseases due to fixation and consumption of complement.
Low levels of total complement (total hemolytic complement: CH50) may occur during infections, disease exacerbation in patients with systemic lupus erythematosus, and in patients with immune complex diseases such as glomerulonephritis.
Undetectable levels suggest the possibility of a complement component deficiency. Individual complement component assays are useful to identify the specific deficiency.