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HelpEpic Code LAB153 C2 Complement, Functional, with Reflex, Serum
Additional Codes
Mayo Code: C2
Epic code: LAB 153
Interface Order Alias: 11635
Cerner: 4357
Performing Laboratory
Mayo Clinic Laboratories in Rochester
Useful For
Investigation of a patient with a low (absent) hemolytic complement, with reflex testing to C3 and C4, if appropriate
Specimen Type
Serum RedOrdering Guidance
This test is for assessment of complement C2 and includes assessment of C3 and C4 as reflex testing. Unless a deficiency has already been identified, initial assessment should begin with the total complement assay (COM / Complement, Total, Serum), which is a screen for suspected complement deficiencies and should be performed 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 but not required
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.
COLLECTION NOTE: Volumes listed are in serum or plasma, draw approximately 2 1/2 times the requested volume in whole blood.
Specimen Minimum Volume
0.5 mL
Specimen Stability Information
| Specimen Type | Temperature | Time | Special Container |
|---|---|---|---|
| Serum Red | Frozen | 21 days |
Reject Due To
| Gross hemolysis | OK |
| Gross lipemia | Reject |
| Gross icterus | OK |
Day(s) Performed
Monday through Friday
Reference Values
25-47 U/mL
Clinical Information
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. This activation process triggers a cascade that includes an amplification loop. The amplification loop in 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, which promotes cell lysis.
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 lytic activity or to clear immune complexes. These patients have increased susceptibility to infections with encapsulated microorganisms. They may also have symptoms that suggest autoimmune disease, of which complement deficiency may be an etiologic factor.
Although rare, C2 deficiency is the most common inherited complement deficiency. Homozygous C2 deficiency has an estimated prevalence ranging from 1 in 10,000 to 1 in 40,000 (the prevalence of heterozygotes is 1 in 100 to 1 in 50). Half of the homozygous patients are clinically normal.
However, discoid lupus erythematosus or systemic lupus erythematosus (SLE) occurs in approximately one-third of patients with homozygous C2 deficiency. Patients with SLE and a C2 deficiency frequently have a normal anti-double stranded DNA titer. Clinically, many have lupus-like skin lesions and photosensitivity, but immunofluorescence studies may fail to demonstrate immunoglobulin or complement along the epidermal-dermal junction.
Other diseases reported to be associated with C2 deficiency include dermatomyositis, glomerulonephritis, vasculitis, atrophoderma, cold urticaria, inflammatory bowel disease, and recurrent infections.
The laboratory findings that suggest C2 deficiency include a hemolytic complement of nearly zero, with normal values for C3 and C4.
Cautions
As with all complement assays, proper sample handling is of utmost importance to ensure that the complement system is not activated before clinical testing.
Absent (or low) C2 functional levels in the presence of normal C2 antigen levels should be replicated with a new serum specimen to confirm that C2 inactivation has not occurred during shipping.
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 (or low) C2 levels in the presence of normal C3 and C4 values are consistent with a C2 deficiency.
Low C2 levels in the presence of low C3 and C4 values are consistent with a complement-consumptive process.
Low C2 and C4 values, in the presence of normal values for C3 is suggestive of C1 esterase inhibitor deficiency.
Reporting Name
C2 Complement,Functional,w/Reflex,SMethod Name
Automated Liposome Lysis Assay
Method Description
C2 complement activity is measured by mixing patient serum with a C2-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 C2. The target liposomes are a commercial reagent (WAKO total complement CH50) and the assay is performed on a Siemens Advia XPT.(Unpublished Mayo method)
CPT Code Information
86161
86160 x 2 (if appropriate)
LOINC Code Information
| Test ID | Test Order Name | Order LOINC Value |
|---|---|---|
| C2 | C2 Complement,Functional,w/Reflex,S | 93977-7 |
| Result ID | Test Result Name | Result LOINC Value |
|---|---|---|
| C2FX | C2 Complement,Functional,S | 93977-7 |
| INT53 | Interpretation | 69048-7 |
Report Available
1 to 3 daysTest 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 Reference
1. Gaither TA, Frank MM: Complement. In: Henry JB, ed. Clinical Diagnosis and Management by Laboratory Methods. 17th ed. WB Saunders Company; 1984:879-892
2. O'Neil KM: Complement deficiency. Clin Rev Allergy Immunol. 2000;19:83-108
3. Frank MM: Complement deficiencies. Pediatr Clin North Am. 2000;47(6):1339-1354
4. Agnello V: Complement deficiency states. Medicine. 1978 Jan;57(1):1-23
5. Buckley D, Barnes L: Childhood subacute cutaneous lupus erythematosus associated with homozygous complement 2 deficiency. Pediatr Dermatol. 1995 Dec;12(4):327-330
6. 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
Reflex Tests
| Test ID | Reporting Name | Available Separately | Always Performed |
|---|---|---|---|
| C4 | Complement C4, S | Yes | No |
| C3 | Complement C3, S | Yes | No |