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Test Code C6FX C6 Complement, Functional, Serum

Reporting Name

C6 Complement, Functional, S

Useful For

Diagnosis of C6 deficiency

 

Investigation of a patient with an undetectable total complement (CH50) level

Method Name

Automated Liposome Lysis Assay

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

Reject Due To

Hemolysis

Mild OK; Gross OK

Lipemia

Mild OK; Gross reject

Icterus

Mild OK; Gross OK

Other

Serum gel tube

Reference Values

32-57 U/mL

Day(s) and Time(s) Performed

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

CPT Code Information

86161

LOINC Code Information

Test ID Test Order Name Order LOINC Value
C6FX C6 Complement, Functional, S 60459-5

 

Result ID Test Result Name Result LOINC Value
C6FX C6 Complement, Functional, S 60459-5

Analytic Time

Same day/1 day

Cautions

The total complement (CH50) assay (COM / Complement, Total, Serum) should be used as a screen for suspected complement deficiencies before ordering individual complement component assays. A deficiency of an individual component of the complement cascade will result in an undetectable total complement level.

 

Absent (or low) C6 functional levels in the presence of normal C6 antigen levels should be replicated with a new serum specimen to confirm that C6 inactivation did not occur during shipping.

Method Description

C6 complement activity is measured by mixing patient serum with a C6-deficient serum. The lytic activity of the serum mixture is tested against sensitized, labeled liposomes. If lysis occurs, the patient serum must be the source of the C6. The target liposomes are a commercial reagent (WAKO total complement CH50), and the assay is performed on a Hitachi 912.(Unpublished Mayo information; Yamamoto S, Kubotsu K, Masaaki K, et al: Automated homogeneous liposome-based assay system for total complement activity. Clin Chem 1995,41:586-590)

Interpretation

Low levels of complement may be due to inherited deficiencies, acquired  deficiencies, or due to complement consumption (eg, as a consequence of infectious or autoimmune processes). Absent C6 levels in the presence of normal C3 and C4 values are consistent with a C6 deficiency. Absent C6 levels in the presence of low C3 and C4 values suggests complement consumption.

 

Normal results indicate both normal C6 protein levels and normal functional activity.

Specimen Retention Time

14 days

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.

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.

 

A number of patients with C6 deficiency have been reported, and the majority of these patients are South African. Most of these patients have systemic meningococcal infection and some have had invasive gonococcal infections. Normal levels of C6 antigen have been reported in patients with dysfunctional C6 lytic activity.