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Test Code MISCLM Atypical Hemolytic Uremic Syndrome (aHUS) Complement Panel, Serum and Plasma

Additional Codes

MAYO Test ID
AHUSD
EPIC Test ID
LAB2608

 

Useful For

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

 

A second-order 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 Complement, Alternate 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
C4D C4d Complement, P No Yes
CBB CBb Complement, P No Yes
SC5B9 SC5b-9 Complement, P No Yes

Reflex Tests

Test ID Reporting Name Available Separately Always Performed
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: Nephelometry

COM3: Automated Liposome Lysis Assay

AH503, C4D, CBB, SC5B9: Enzyme-Linked Immunosorbent Assay (ELISA)

Reporting Name

aHUS Complement Panel, S and P

Specimen Type

Plasma Na Cit
Serum Red


Advisory Information


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



Specimen Required


Both plasma and serum are required for this test.

 

Patient Preparation: Fasting preferred

 

Specimen Type: Plasma

Collection Container/Tube: Light-blue top (3.2% sodium citrate)

Submission Container/Tube: Plastic vial

Specimen Volume: 1.5 mL

Collection Instructions:

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

2. Spin down and separate plasma from cells; 1,500 x g for 10 minutes at 4° C.

3. Freeze specimen within 30 minutes.

 

Specimen Type: Serum

Collection Container/Tube: Red top

Submission Container/Tube: Plastic vial

Specimen Volume: 1.5 mL

Collection Instructions:

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

2. Spin down and separate serum from clot.

3. Freeze specimen within 30 minutes.


Specimen Minimum Volume

Serum, Plasma: 1 mL

Specimen Stability Information

Specimen Type Temperature Time
Plasma Na Cit Frozen 14 days
Serum Red Frozen 14 days

Reject Due To

Hemolysis

Mild OK; Gross OK

Lipemia

Mild OK; Gross reject

Icterus

NA

Other

Serum gel tube, heparin plasma

Reference Values

FACTOR B COMPLEMENT ANTIGEN

15.2-42.3 mg/dL

 

SC5b-9 COMPLEMENT

≤250 ng/mL

 

FACTOR H COMPLEMENT ANTIGEN

23.6-43.1 mg/dL

 

C4d COMPLEMENT ACTIVATION FRAGMENT

≤9.8 mcg/mL

 

CBb COMPLEMENT ACTIVATION FRAGMENT

≤1.6 mcg/mL

 

COMPLEMENT C4

14-40 mg/dL

 

COMPLEMENT C3

75-175 mg/dL

 

COMPLEMENT, ALTERNATE PATHWAY (AH50), FUNCTIONAL

≥46% normal

 

COMPLEMENT, TOTAL

≥16 years: 30-75 U/mL

Reference values have not been established for patients who are <16 years of age.

Day(s) and Time(s) Performed

Varies

Analytic Time

2 days

Performing Laboratory

Mayo Medical Laboratories in Rochester

CPT Code Information

86160 x 7-Complement; antigen, each component

86161-functional activity, each component

86162-total hemolytic (CH50)

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
62584 C4d Complement, P 39565-7
62585 CBb Complement, P 4517-9
FBCA Factor B Complement Antigen, S In Process
FHCA Factor H Complement Antigen, S In Process
62586 SC5b-9 Complement, P In Process
38316 Complement, Alternate 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 In Process
ECPRO Is patient receiving Eculizumab? In Process