Atypical Hemolytic Uremic Syndrome Complement Panel, Serum and Plasma
Use
This test is used for detecting deficiencies in the alternative pathway that can cause atypical Hemolytic Uremic Syndrome (aHUS), dense deposit disease, and C3 glomerulonephritis. It serves as a second-tier test aiding in the differential diagnosis of thrombotic microangiopathies (TMA), a group of disorders characterized by thrombosis in small blood vessels, which leads to microangiopathic hemolytic anemia, thrombocytopenia, and organ damage. Identifying the complement system dysfunction helps in the effective management of these conditions, especially in differentiating aHUS from other forms of TMA, thus avoiding unnecessary treatments and facilitating targeted therapies.
Special Instructions
This test should be performed before treatment initiation and in the absence of therapy with complement inhibitors, such as eculizumab or ravulizumab, as these inhibitors affect the assays' performance. It is recommended as a second-tier test for evaluating TMA and is guided by specific protocols for patients on eculizumab or ravulizumab.
Limitations
The test is not suitable for use with specimens collected after plasma exchange within 48 hours. The handling of the specimen is critical to prevent in vitro activation of the complement system, otherwise, the results might not be reliable. Complement inhibitors reduce the utility of these assays; therefore, testing should coincide with times of inhibitor absence if possible.
New York Approved
Methodology
Immunoassay (ELISA)
Biomarkers
LOINC Codes
- 34547-0
- 4517-9
- 2269-9
- 4519-5
- 93244-2
- 74520-8
- 4532-8
- 4485-9
- 4498-2
- 69048-7
- 86955-2
Result Turnaround Time
12-21 days
Related Documents
For more information, please review the documents below
Specimen
Serum
Volume
1.5 mL total in 3 separate plastic vials, each containing 0.5 mL
Minimum Volume
1.5 mL
Container
Plastic vial
Collection Instructions
Immediately place the tube on wet ice, allow the specimen to clot, centrifuge at 4°C, aliquot serum into 3 separate plastic vials, freeze within 30 minutes of centrifugation.
Patient Preparation
Fasting for 12 hours preferred but not required; do not collect within 48 hours following plasma exchange.
Causes for Rejection
Gross hemolysis, gross lipemia
Stability Requirements
| Temperature | Period |
|---|---|
| Frozen | 14 days |