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Test Code AHUSD Atypical Hemolytic Uremic Syndrome Complement Panel, Serum and Plasma

Useful For

Detecting deficiencies in the alternative pathway that can cause atypical-hemolytic uremic syndrome, dense deposit disease, and C3 glomerulonephritis

 

A second-tier test that aids in the differential diagnosis of thrombotic microangiopathies

Profile Information

Test ID Reporting Name Available Separately Always Performed
INTGA AHUS Interpretation No Yes
COM3 Complement, Total, S Yes, (order COM) Yes
AH503 Alternative Complement Path Func, S Yes, (order AH50) Yes
C3HUS Complement C3, S Yes, (order C3) Yes
C4HUS Complement C4, S Yes, (order C4) Yes
FBCA Factor B Complement Antigen, S No Yes
FHCA Factor H Complement Antigen, S No Yes
CBB CBb Complement, P No Yes
SC5B9 SC5b-9 Complement, P Yes, (C5B9) Yes

Reflex Tests

Test ID Reporting Name Available Separately Always Performed
C1Q Complement C1q, S Yes No
C1QFX C1Q Complement, Functional, S Yes No
C2FXN C2 Complement, Functional, S, NR Yes No
C3FX C3 Complement, Functional, S Yes No
C4FX C4 Complement, Functional, S Yes No
C5FX C5 Complement, Functional, S Yes No
C6FX C6 Complement, Functional, S Yes No
C7FX C7 Complement, Functional, S Yes No
C8FX C8 Complement, Functional, S Yes No
C9FX C9 Complement, Functional, S Yes No
C5AG2 C5 Complement, Antigen, S Yes, (order C5AG) No

Method Name

C3HUS, C4HUS, FBCA, FHCA; C5AG2: Nephelometry

COM3: Automated Liposome Lysis Assay

AH503, CBB, SC5B9: Enzyme-Linked Immunosorbent Assay (ELISA)

INTGA: Medical Interpretation

Reporting Name

aHUS Complement Panel, S and P

Specimen Type

Plasma EDTA
Serum


Ordering Guidance


This test should be performed prior to treatment initiation and in the absence of therapy with complement inhibitors, such as eculizumab or ravulizumab. Complement inhibitors will affect performance of these assays.

 

For evaluating patients with possible thrombotic microangiopathies (TMA), the recommended first-tier test is ADM13 / ADAMTS13 Activity and Inhibitor Profile, Plasma. This test should be a second-tier test for TMA.

 

For patients who have received eculizumab or need to monitor response to eculizumab therapy, the recommended test is ECMP / Eculizumab Monitoring Panel, Serum. Soluble membrane attack complex should not be used as a standalone assay to monitor eculizumab efficiency.

 

For patients who have received ravulizumab or need to monitor response to ravulizumab therapy, the recommended test is RAVMP / Ravulizumab Monitoring Panel, Serum. Soluble membrane attack complex (sMAC) should not be used as a standalone assay to monitor ravulizumab efficiency.



Specimen Required


Both serum and plasma are required for this test.

 

Patient Preparation:

1. Fasting preferred but not required.

2. Do not collect specimens for at least 48 hours following plasma exchange.

Supplies: Sarstedt Aliquot Tube, 5 mL (T914)

 

Specimen Type: Serum

Collection Container/Tube:

Preferred: Serum gel

Acceptable: Red top

Submission Container/Tube: Plastic vial

Specimen Volume: 1.5 mL total in 3 separate plastic vials, each containing 0.5 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 3 separate plastic vials, each containing 0.5 mL.

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 Type: Plasma

Collection Container/Tube:

Preferred: Lavender top (K2 EDTA)

Acceptable: Lavender top (K3 EDTA), light-blue top (sodium citrate)

Submission Container/Tube: Plastic vial

Specimen Volume: 1.5 mL total in 2 separate plastic vials, each containing 0.75 mL

Collection Instructions:

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

2. Centrifuge between 1000 and 2000 x g for 10 minutes at 4° C and aliquot plasma into 2 separate plastic vials, each containing 0.75 mL.

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 plasma is aliquoted and frozen.


Specimen Minimum Volume

Serum: 1.5 mL; plasma: 1.5 mL

Specimen Stability Information

Specimen Type Temperature Time Special Container
Plasma EDTA Frozen 14 days
Serum Frozen 14 days

Reference Values

FACTOR B COMPLEMENT ANTIGEN

15.2-42.3 mg/dL

 

SC5b-9 COMPLEMENT

≤250 ng/mL

 

FACTOR H COMPLEMENT ANTIGEN

18.5 to 40.8 mg/dL

 

CBb COMPLEMENT ACTIVATION FRAGMENT

≤1.6 mcg/mL

 

COMPLEMENT C4

14-40 mg/dL

 

COMPLEMENT C3

75-175 mg/dL

 

ALTERNATIVE COMPLEMENT, PATHWAY (AH50) FUNCTIONAL

≥46% Normal

 

COMPLEMENT, TOTAL

30-75 U/mL

Day(s) Performed

Varies

Report Available

12 to 21 days

Performing Laboratory

Mayo Clinic Laboratories in Rochester

CPT Code Information

86160 x 6

86161

86162

LOINC Code Information

Test ID Test Order Name Order LOINC Value
AHUSD aHUS Complement Panel, S and P In Process

 

Result ID Test Result Name Result LOINC Value
62585 CBb Complement, P 4517-9
FBCA Factor B Complement Antigen, S 2269-9
FHCA Factor H Complement Antigen, S 4519-5
62586 SC5b-9 Complement, P 93244-2
38316 Alternative Complement Path Func, S 74520-8
COM3 Complement, Total, S 4532-8
C3HUS Complement C3, S 4485-9
C4HUS Complement C4, S 4498-2
39844 AHUS Interpretation 69048-7
ECPRO Is Eculizumab or Ravulizumab taken? 86955-2

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.

Forms

If not ordering electronically, complete, print, and send 1 of the following forms with the specimen:

-Renal Diagnostics Test Request (T830)

-Coagulation Test Request (T753)