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26476 Atypical Hemolytic Uremic Syndrome Complement Panel, Serum and Plasma (AHUSD)

Atypical Hemolytic Uremic Syndrome Complement Panel, Serum and Plasma (AHUSD)
Test Code: AHUSDSO
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

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 ECUMP / Eculizumab Monitoring Panel, Serum. Soluble membrane attack complex (sMAC) should not be used as a standalone assay to monitor eculizumab efficiency.

Specimen Requirements
Fasting RequiredSpecimen TypePreferred Container/TubeAcceptable Container/TubeSpecimen VolumeSpecimen Minimum Volume
(allows for 1 repeat)		Pediatric Minimum Volume
(no repeat)
**Both Plasma and Serum are required for this test**​ ​ ​​​​​
​Preferred​Plasma​Light-Blue Top (3.2% sodium citrate)​​1.5 mL​1 mL​
​Preferred​Serum​Red Top Tube (RTT)​​1.5 mL​1 mL​
Collection Processing Instructions

Patient Preparation:
1. Fasting preferred.
2. Samples should not be collected earlier than 48 hours following plasma exchange.

​Plasma:

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

2. Centrifuge; 1500 x g for 10 minutes at 4 degrees C and aliquot plasma into plastic vial.

3. Freeze specimen within 30 minutes.

Serum:

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

2. Centrifuge at 4 degrees C and aliquot serum into 5 mL plastic vial.

3. Freeze specimen within 30 minutes.

Specimen Stability Information
Specimen TypeTemperatureTime
​Plasma Na Cit​Frozen (preferred)​14 days
​Serum Red​Frozen (preferred)​14 days
Rejection Criteria
Gross lipemia
Interference

​As with all complement assays, proper sample handling is of utmost importance to ensure that the complement system is not activated before clinical testing.

Performing Laboratory Information
Performing LocationDay(s) Test PerformedReport AvailableMethodology/Instrumentation
​Mayo Clinic Laboratories​Varies​12 to 21 days​

C3HUS, C4HUS, FBCA, FHCA: Nephelometry

COM3: Automated Liposome Lysis Assay

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

Reference Lab
Mayo Clinic Laboratories
Reference Range Information
Performing LocationReference Range
​Mayo Clinic Laboratories​

FACTOR B COMPLEMENT ANTIGEN

15.2-42.3 mg/dL

SC5b-9 COMPLEMENT

< or =250 ng/mL

FACTOR H COMPLEMENT ANTIGEN

18.5 to 40.8 mg/dL

C4d COMPLEMENT ACTIVATION FRAGMENT

< or =9.8 mcg/mL

CBb COMPLEMENT ACTIVATION FRAGMENT

< or =1.6 mcg/mL

COMPLEMENT C4

14-40 mg/dL

COMPLEMENT C3

75-175 mg/dL

ALTERNATIVE COMPLEMENT, PATHWAY (AH50) FUNCTIONAL

> or =46% normal

COMPLEMENT, TOTAL

30-75 U/mL

Interpretation

​An interpretive report will be provided.

Outreach CPTs
CPTModifier
(if needed)		QuantityDescriptionComments
​86160​​7​​
​86161​​1​​
​86162​​1​​
​86160​​1​C1Q​if needed
​86161​​1​C1QFX​if needed
​86161​​1​C2FXN​if needed
​86161​​1​C3FX​if needed
​86161​​1​C4FX​if needed
​86161​​1​C5FX​if needed
​86161​​1​C6FX​if needed
​86161​​1​C7FX​if needed
​86161​​1​C8FX​if needed
​86161​​1​C9FX​if needed
​86160​​1​C5AG2​if needed
Ordering Applications
Ordering ApplicationDescription
​COM​Atyp HeomlyticUremicSynd (aHUS) Comp (AHUSD)
​Cerner​Aypical Hemolytic Uremic Syndrome (aHUS) Complement Panel, Serum and Plasma (AHUSD)
If the ordering application you are looking for is not listed, contact your local laboratory for assistance.
Specimen Requirements
Fasting RequiredSpecimen TypePreferred Container/TubeAcceptable Container/TubeSpecimen VolumeSpecimen Minimum Volume
(allows for 1 repeat)		Pediatric Minimum Volume
(no repeat)
**Both Plasma and Serum are required for this test**​ ​ ​​​​​
​Preferred​Plasma​Light-Blue Top (3.2% sodium citrate)​​1.5 mL​1 mL​
​Preferred​Serum​Red Top Tube (RTT)​​1.5 mL​1 mL​
Collection Processing

Patient Preparation:
1. Fasting preferred.
2. Samples should not be collected earlier than 48 hours following plasma exchange.

​Plasma:

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

2. Centrifuge; 1500 x g for 10 minutes at 4 degrees C and aliquot plasma into plastic vial.

3. Freeze specimen within 30 minutes.

Serum:

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

2. Centrifuge at 4 degrees C and aliquot serum into 5 mL plastic vial.

3. Freeze specimen within 30 minutes.

Specimen Stability Information
Specimen TypeTemperatureTime
​Plasma Na Cit​Frozen (preferred)​14 days
​Serum Red​Frozen (preferred)​14 days
Rejection Criteria
Gross lipemia
Interference

​As with all complement assays, proper sample handling is of utmost importance to ensure that the complement system is not activated before clinical testing.

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

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 ECUMP / Eculizumab Monitoring Panel, Serum. Soluble membrane attack complex (sMAC) should not be used as a standalone assay to monitor eculizumab efficiency.

Reference Range Information
Performing LocationReference Range
​Mayo Clinic Laboratories​

FACTOR B COMPLEMENT ANTIGEN

15.2-42.3 mg/dL

SC5b-9 COMPLEMENT

< or =250 ng/mL

FACTOR H COMPLEMENT ANTIGEN

18.5 to 40.8 mg/dL

C4d COMPLEMENT ACTIVATION FRAGMENT

< or =9.8 mcg/mL

CBb COMPLEMENT ACTIVATION FRAGMENT

< or =1.6 mcg/mL

COMPLEMENT C4

14-40 mg/dL

COMPLEMENT C3

75-175 mg/dL

ALTERNATIVE COMPLEMENT, PATHWAY (AH50) FUNCTIONAL

> or =46% normal

COMPLEMENT, TOTAL

30-75 U/mL

Interpretation

​An interpretive report will be provided.

For more information visit:
Performing Laboratory Information
Performing LocationDay(s) Test PerformedReport AvailableMethodology/Instrumentation
​Mayo Clinic Laboratories​Varies​12 to 21 days​

C3HUS, C4HUS, FBCA, FHCA: Nephelometry

COM3: Automated Liposome Lysis Assay

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

Reference Lab
Mayo Clinic Laboratories
For billing questions, see Contacts
Outreach CPTs
CPTModifier
(if needed)		QuantityDescriptionComments
​86160​​7​​
​86161​​1​​
​86162​​1​​
​86160​​1​C1Q​if needed
​86161​​1​C1QFX​if needed
​86161​​1​C2FXN​if needed
​86161​​1​C3FX​if needed
​86161​​1​C4FX​if needed
​86161​​1​C5FX​if needed
​86161​​1​C6FX​if needed
​86161​​1​C7FX​if needed
​86161​​1​C8FX​if needed
​86161​​1​C9FX​if needed
​86160​​1​C5AG2​if needed
For most current information refer to the Marshfield Laboratory online reference manual.