Detecting deficiencies in the alternative pathway that can cause atypical-hemolytic uremic syndrome, dense deposit disease, and C3 glomerulonephritis
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 ECUMP / Eculizumab Monitoring Panel, Serum. Soluble membrane attack complex (sMAC) should not be used as a standalone assay to monitor eculizumab efficiency.
Specimen Type
Blood
Container
Lavender Top Tube
Collection Instructions
Serum and Plasma are required for this test Container/Tube: Both Lavender Top AND Red Top Tube Specimen: 1.5 mL citrated plasma ( 1 mL min) AND 1.5 mL serum (1 mL min) Collection Instructions: 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 from Lavender tube and serum from the Red tube into plastic vial. 3. Freeze specimen within 30 minutes.
Patient Preparation: 1. Fasting preferred. 2. Samples should not be collected earlier than 48 hours following plasma exchange. Alternate Tube: Plasma : Light Blue (3.3 % sodium citrate) Serum Gold Top Tube
