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Test Code C5FX C5 Complement, Functional, Serum

Reporting Name

C5 Complement, Functional, S

Useful For

Diagnosis of C5 deficiency

 

Investigation of a patient with an undetectable total complement level

Method Name

Automated Liposome Lysis Assay

Performing Laboratory

Mayo Clinic Laboratories in Rochester

Specimen Type

Serum


Ordering Guidance


The total complement 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.



Specimen Required


Patient Preparation: Fasting preferred

Supplies: Sarstedt Aliquot Tube, 5 mL (T914)

Collection Container/Tube:

Preferred: Serum gel

Acceptable: Red top

Submission Container/Tube: Plastic vial

Specimen Volume: 1 mL

Collection Instructions:

1. Immediately after specimen collection, place the tube on wet ice and allow specimen to clot.

2. Centrifuge at 4° C and aliquot serum into a plastic vial.

3. Within 30 minutes of centrifugation, freeze specimen. Sample must be placed on dry ice if not frozen immediately.

NOTE: If a refrigerated centrifuge is not available, it is acceptable to use a room temperature centrifuge, provided the sample is kept on ice before centrifugation, and immediately afterward, the serum is aliquoted and frozen.


Specimen Minimum Volume

0.5 mL

Specimen Stability Information

Specimen Type Temperature Time Special Container
Serum Frozen 14 days

Reject Due To

Gross hemolysis OK
Gross lipemia Reject
Gross icterus OK

Reference Values

29-53 U/mL

Day(s) Performed

Monday through Friday

CPT Code Information

86161

LOINC Code Information

Test ID Test Order Name Order LOINC Value
C5FX C5 Complement, Functional, S 60472-8

 

Result ID Test Result Name Result LOINC Value
C5FX C5 Complement, Functional, S 60472-8

Test Classification

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

Clinical Information

Complement proteins are components of the innate immune system. There are 3 pathways to complement activation: 1) the classical pathway, 2) the alternative (or properdin) pathway, and 3) the lectin (mannan-binding lectin) pathway. The classical pathway of the complement system is composed of a series of proteins that are activated in response to the presence of immune complexes. A single IgM molecule or 2 IgG molecules are sufficient to trigger activation of the recognition complex initiated by C1q. The activation process triggers a cascade that includes an amplification loop. The amplification loop is mediated by C3, with cleavage of a series of proteins, and results in 3 main end products: 1) anaphylatoxins that promote inflammation (C3a, C5a), 2) opsonization peptides that are chemotactic for neutrophils (C3b) and facilitate phagocytosis, and 3) the membrane attack complex (MAC), which promotes cell lysis.

 

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. There have been between 20 and 100 cases of C5 deficiency reported.

 

Complement levels can be detected by antigen assays that quantitate the amount of the protein (C5AG / C5 Complement, Antigen, Serum). For most of the complement proteins, a small number of cases have been described in which the protein is present but is nonfunctional. These rare cases require a functional assay to detect the deficiency.

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 C5 levels in the presence of normal C3 and C4 values are consistent with a C5 deficiency. Absent C5 levels in the presence of low C3 and C4 values suggest complement consumption.

 

Normal results indicate both normal C5 protein levels and normal functional activity.

Cautions

As with all complement assays, proper specimen handling is of utmost importance to ensure that the complement system is not activated before clinical testing.

 

Absent (or low) complement C5 functional levels in the presence of normal C5 antigen levels should be replicated with a new serum specimen to confirm that C5 inactivation did not occur during shipping.

Method Description

Activity of C5 is measured by mixing patient serum with a C5-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 C5. The target liposomes are a commercial reagent (WAKO total complement CH50), and the assay is performed on a Siemens Advia XPT.(Unpublished Mayo method)

Report Available

1 to 3 days

Specimen Retention Time

14 days