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Mayo Test ID C5FX C5 Complement, Functional, 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 5 mL Aliquot Tube (T914)

Collection Container/Tube: Red top

Submission Container/Tube: Plastic vial

Specimen Volume: 1 mL

Collection Instructions:

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

2. Centrifuge and aliquot serum into plastic vial.

3. Immediately freeze specimen.


Useful For

Diagnosis of C5 deficiency

 

Investigation of a patient with an undetectable total complement level

Method Name

Automated Liposome Lysis Assay

Reporting Name

C5 Complement, Functional, S

Specimen Type

Serum Red

Specimen Minimum Volume

0.5 mL

Specimen Stability Information

Specimen Type Temperature Time Special Container
Serum Red Frozen 14 days

Reject Due To

Gross hemolysis OK
Gross lipemia Reject
Gross icterus OK

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 (or 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. Approximately 40 cases of C5 deficiency have been 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.

Reference Values

29-53 U/mL

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

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.

Clinical Reference

1. Sonntag J, Brandenburg U, Polzehl D, et al: Complement systems in healthy term newborns: reference values in umbilical cord blood. Pediatr Dev Pathol. 1998 Mar-Apr;1(2):131-135

2. Prellner K, Sjoholm AG, Truedsson L: Concentrations of C1q, factor B, factor D and properdin in healthy children, and the age-related presence of circulating C1r-C1s complexes. Acta Paediatr Scand. 1987 Nov;76(6):939-943

3. Davis ML, Austin C, Messmer BL, et al: IFCC-standardization pediatric reference intervals for 10 serum proteins using the Beckman Array 360 system. Clin Biochem. 1996 Oct;29,5:489-492

4. Gaither TA, Frank MM: Complement. In: Henry JB, ed. Clinical Diagnosis and Management by Laboratory Methods. 17th ed. WB Saunders Company; 1984:879-892

5. O'Neil KM: Complement deficiency. Clin Rev Allergy Immunol. 2000 Oct;19:83-108

6. Frank MM: Complement deficiencies. Pediatr Clin North Am. 2000 Dec;47(6):1339-1354

7. Willrich MAV, Braun KMP, Moyer AM, Jeffrey DH, Frazer-Abel A. Complement testing in the clinical laboratory. Crit Rev Clin Lab Sci. 2021 Nov;58(7):447-478. doi: 10.1080/10408363.2021.1907297

Method Description

C5 complement activity 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)

Day(s) Performed

Monday through Friday

Report Available

1 to 3 days

Specimen Retention Time

14 days

Performing Laboratory

Mayo Clinic Laboratories in Rochester

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.

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