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Test Code C9 C9 Complement, Functional, Serum

Additional Codes

Mayo Test ID
C9FX

Reporting Name

C9 Complement, Functional, S

Useful For

Diagnosis of C9 deficiency

 

Investigation of a patient with a low total (hemolytic) complement (CH50) level

Performing Laboratory

Mayo Medical Laboratories in Rochester

Specimen Type

Serum Red


Specimen Required


Collection Container/Tube: Red top

Submission Container/Tube: Plastic vial

Specimen Volume: 1 mL

Collection Instructions:

1. Immediately after drawing the specimen, place the tube on wet ice.

2. Spin down and separate serum from clot.

3. Immediately freeze specimen.

Additional Information: Fasting preferred.


Specimen Minimum Volume

0.5 mL

Specimen Stability Information

Specimen Type Temperature Time
Serum Red Frozen 14 days

Reference Values

37-61 U/mL

Day(s) and Time(s) Performed

Monday through Saturday; Continuous with a 3 p.m. cutoff

Test Classification

This test was developed and its performance characteristics determined by Mayo Clinic in a manner consistent with CLIA requirements. This test has not been cleared or approved by the U.S. Food and Drug Administration.

CPT Code Information

86161

LOINC Code Information

Test ID Test Order Name Order LOINC Value
C9FX C9 Complement, Functional, S 87727-4

 

Result ID Test Result Name Result LOINC Value
C9FX C9 Complement, Functional, S 87727-4

Clinical Information

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 (mannan-binding protein [MBP]) 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).

 

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.

 

C9 deficiency is common in the Japanese population and has been reported to occur in almost 1% of the population. The lytic activity of C9-deficient serum is decreased. However, the assembly of C5b-C8 complexes will result in a transmembrane channel with lytic activity, although the lytic activity is reduced. Many C9-deficient patients are therefore asymptomatic. C9-deficient patients may, however, present with invasive neisserial infections.

 

Complement levels can be detected by antigen assays that quantitate the amount of the protein. For most of the complement proteins, a small number of cases have been described in which the protein is present but is non functional. These rare cases require a functional assay to detect the deficiency.

Analytic Time

Same day/1 day

NY State Approved

Yes

Method Name

Automated Liposome Lysis Assay

Northwell Health Laboratories Additional Information:

Computer Interface Code

   PDM # 5903300