Test Catalog

Test Id : C2

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C2 Complement, Functional, with Reflex, Serum

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Overview

Test Catalog

Useful For
Suggests clinical disorders or settings where the test may be helpful

Investigation of a patient with a low (absent) hemolytic complement, with reflex testing to C3 and C4, if appropriate

Reflex Tests
Lists tests that may or may not be performed, at an additional charge, depending on the result and interpretation of the initial tests.

Test Id Reporting Name Available Separately Always Performed
C4 Complement C4, S Yes No
C3 Complement C3, S Yes No

Testing Algorithm
Delineates situations when tests are added to the initial order. This includes reflex and additional tests.

If the C2 result is less than 15 U/mL, then complement C3 and C4 will be performed at an additional charge.

Method Name
A short description of the method used to perform the test

Automated Liposome Lysis Assay

NY State Available
Indicates the status of NY State approval and if the test is orderable for NY State clients.

Yes

Reporting Name
Lists a shorter or abbreviated version of the Published Name for a test

C2 Complement,Functional,w/Reflex,S

Aliases
Lists additional common names for a test, as an aid in searching

C2 Functional

Testing Algorithm
Delineates situations when tests are added to the initial order. This includes reflex and additional tests.

If the C2 result is less than 15 U/mL, then complement C3 and C4 will be performed at an additional charge.

Specimen Type
Describes the specimen type validated for testing

Serum Red

Ordering 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
Defines the optimal specimen required to perform the test and the preferred volume to complete testing

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.

Specimen Minimum Volume
Defines the amount of sample necessary to provide a clinically relevant result as determined by the testing laboratory. The minimum volume is sufficient for one attempt at testing.

0.5 mL

Reject Due To
Identifies specimen types and conditions that may cause the specimen to be rejected

Gross hemolysis OK
Gross lipemia Reject
Gross icterus OK

Specimen Stability Information
Provides a description of the temperatures required to transport a specimen to the performing laboratory, alternate acceptable temperatures are also included

Specimen Type Temperature Time Special Container
Serum Red Frozen 21 days

Useful For
Suggests clinical disorders or settings where the test may be helpful

Investigation of a patient with a low (absent) hemolytic complement, with reflex testing to C3 and C4, if appropriate

Testing Algorithm
Delineates situations when tests are added to the initial order. This includes reflex and additional tests.

If the C2 result is less than 15 U/mL, then complement C3 and C4 will be performed at an additional charge.

Clinical Information
Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test

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.

Reference Values
Describes reference intervals and additional information for interpretation of test results. May include intervals based on age and sex when appropriate. Intervals are Mayo-derived, unless otherwise designated. If an interpretive report is provided, the reference value field will state this.

25-47 U/mL

Interpretation
Provides information to assist in interpretation of the test results

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.

Cautions
Discusses conditions that may cause diagnostic confusion, including improper specimen collection and handling, inappropriate test selection, and interfering substances

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.

Clinical Reference
Recommendations for in-depth reading of a clinical nature

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

Method Description
Describes how the test is performed and provides a method-specific reference

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)

PDF Report
Indicates whether the report includes an additional document with charts, images or other enriched information

No

Day(s) Performed
Outlines the days the test is performed. This field reflects the day that the sample must be in the testing laboratory to begin the testing process and includes any specimen preparation and processing time before the test is performed. Some tests are listed as continuously performed, which means that assays are performed multiple times during the day.

Monday through Friday

Report Available
The interval of time (receipt of sample at Mayo Clinic Laboratories to results available) taking into account standard setup days and weekends. The first day is the time that it typically takes for a result to be available. The last day is the time it might take, accounting for any necessary repeated testing.

1 to 3 days

Specimen Retention Time
Outlines the length of time after testing that a specimen is kept in the laboratory before it is discarded

14 days

Performing Laboratory Location
Indicates the location of the laboratory that performs the test

Rochester

Fees :
Several factors determine the fee charged to perform a test. Contact your U.S. or International Regional Manager for information about establishing a fee schedule or to learn more about resources to optimize test selection.

  • Authorized users can sign in to Test Prices for detailed fee information.
  • Clients without access to Test Prices can contact Customer Service 24 hours a day, seven days a week.
  • Prospective clients should contact their account representative. For assistance, contact Customer Service.

Test Classification
Provides information regarding the medical device classification for laboratory test kits and reagents. Tests may be classified as cleared or approved by the US Food and Drug Administration (FDA) and used per manufacturer instructions, or as products that do not undergo full FDA review and approval, and are then labeled as an Analyte Specific Reagent (ASR) product.

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
Provides guidance in determining the appropriate Current Procedural Terminology (CPT) code(s) information for each test or profile. The listed CPT codes reflect Mayo Clinic Laboratories interpretation of CPT coding requirements. It is the responsibility of each laboratory to determine correct CPT codes to use for billing.

CPT codes are provided by the performing laboratory.

86161

86160 x 2 (if appropriate)

LOINC® Information
Provides guidance in determining the Logical Observation Identifiers Names and Codes (LOINC) values for the order and results codes of this test. LOINC values are provided by the performing laboratory.

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
Applies only to results expressed in units of measure originally reported by the performing laboratory. These values do not apply to results that are converted to other units of measure.
C2FX C2 Complement,Functional,S 93977-7
INT53 Interpretation 69048-7

Test Setup Resources

Setup Files
Test setup information contains test file definition details to support order and result interfacing between Mayo Clinic Laboratories and your Laboratory Information System.

Excel | Pdf

Sample Reports
Normal and Abnormal sample reports are provided as references for report appearance.

Normal Reports | Abnormal Reports

SI Sample Reports
International System (SI) of Unit reports are provided for a limited number of tests. These reports are intended for international account use and are only available through MayoLINK accounts that have been defined to receive them.

SI Normal Reports | SI Abnormal Reports