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HelpTest Code ECMP Eculizumab Monitoring Panel, Serum
Ordering Guidance
To measure only serum concentration of eculizumab, order ECULI / Eculizumab, Serum.
Specimen Required
Patient Preparation:
1. Fasting preferred.
2. Suggest discontinuing natalizumab at least 4 weeks prior to testing for eculizumab quantitation in serum. Patient should consult the healthcare provider who prescribed this drug to determine if discontinuation is an option. If not, ok to proceed with testing while taking natalizumab.
Supplies: Sarstedt Aliquot Tube, 5 mL (T914)
Collection Container/Tube: Red top (serum gel/SST are not acceptable)
Submission Container/Tube: 2 Plastic vials
Specimen Volume: 2 mL in 2 plastic vials, each vial containing 1 mL
Collection Instructions:
1. Draw blood immediately before next scheduled dose.
2. Immediately after specimen collection, place the tube on wet ice.
3. After sample has clotted on wet ice, centrifuge at 4° C and aliquot serum into two 5 mL plastic vials.
4. Freeze specimen within 30 minutes of centrifugation. Sample must be placed on dry ice if not frozen immediately.
Useful For
Monitoring of complement blockage by eculizumab
Assessing the response to eculizumab therapy
Assessing the need for dose escalation
Evaluating the potential for dose de-escalation or discontinuation of therapy in remission states
Monitoring patients who need to be above a certain eculizumab concentration to improve the odds of a clinical response for therapy optimization
This test is not useful as the sole basis for a diagnosis or treatment decisions.
Profile Information
| Test ID | Reporting Name | Available Separately | Always Performed |
|---|---|---|---|
| ECULI | Eculizumab, S | Yes | Yes |
| EAH50 | Eculizumab Complement Blockage, S | No | Yes |
| ECUIN | Eculizumab Interpretation, S | No | Yes |
Method Name
EAH50: Enzyme-Linked Immunosorbent Assay (ELISA)
ECULI: Liquid Chromatography Tandem Mass Spectrometry (LC-MS/MS), High Resolution Accurate Mass
Reporting Name
Eculizumab Monitoring Panel, SSpecimen Type
SerumSerum Red
Specimen Minimum Volume
1 mL total in 2 plastic vials, each vial containing 0.5 mL
Specimen Stability Information
| Specimen Type | Temperature | Time | Special Container |
|---|---|---|---|
| Serum | Frozen | 14 days | |
| Serum Red | Frozen | 14 days |
Reference Values
Eculizumab Complement Blockage:
≥46% normal
Eculizumab:
Lower limit of quantitation =5.0 mcg/mL
>35 Therapeutic concentration for paroxysmal nocturnal hemoglobinuria (PNH)
>50 Therapeutic concentration for atypical hemolytic uremic syndrome (aHUS)
Interpretation
Minimum trough therapeutic concentrations (immediately before next infusion) of eculizumab are expected to be above 35 mcg/mL for paroxysmal nocturnal hemoglobinuria and above 50 mcg/mL for atypical hemolytic uremic syndrome.
For the complement blockage monitoring of eculizumab:
-When eculizumab is present in serum at concentrations around 100 mcg/mL, the results are below the limit of quantitation of the assay (<10% of normal).
Clinical Reference
1. Frazer-Abel A, Sepiashvili L, Mbughuni MM, Willrich MA. Overview of laboratory testing and clinical presentations of complement deficiencies and dysregulation. Adv Clin Chem. 2016;77:1-75. doi:10.1016/bs.acc.2016.06.001
2. Go RS, Winters JL, Leung N, et al. Thrombotic microangiopathy care pathway: A consensus statement for the Mayo Clinic Complement Alternative Pathway-Thrombotic Microangiopathy (CAP-TMA) Disease-Oriented Group. Mayo Clin Proc. 2016;91(9):1189-1211. doi:10.1016/j.mayocp.2016.05.015
3. Willrich MAV, Andreguetto BD, Sridharan M, et al: The impact of eculizumab on routine complement assays. J Immunol Methods. 2018;460:63-71. doi:10.1016/j.jim.2018.06.010
4. Ardissino G, Tel F, Sgarbanti M, et al. Complement functional tests for monitoring eculizumab treatment in patients with atypical hemolytic uremic syndrome: an update. Pediatr Nephrol. 2018;33(3):457-461
5. Volokhina EB, van de Kar NC, Bergseth G, et al. Sensitive, reliable and easy-performed laboratory monitoring of eculizumab therapy in atypical hemolytic uremic syndrome. Clin Immunol. 2015;160(2):237-243
6. Sridharan M, Willrich MA, Go RS. Personalized Dosing of Eculizumab Using C5 Functional Activity and Eculizumab Level in Complement-mediated Thrombotic Microangiopathy: A Safe and Cost-saving Approach. XXVIII Congress of the International Society on Thrombosis and Haemostasis. Virtual ISTH 2020; July 12-14, 2020.
7. Cataland S, Ariceta G, Chen P, et al. Discordance between free C5 and CH50 complement assays in measuring complement C5 inhibition in patients with aHUS treated with ravulizumab. Blood. 2019;134(Supplement_1):1099
8. Willrich MA, Murray DL, Barnidge DR, Ladwig PM, Snyder MR. Quantitation of infliximab using clonotypic peptides and selective reaction monitoring by LC-MS/MS. Int Immunopharmacol. 2015;28(1):513-520. doi:10.1016/j.intimp.2015.07.007
9. Ladwig PM, Barnidge DR, Willrich MA. Quantification of the IgG2/4 kappa monoclonal therapeutic eculizumab from serum using isotype specific affinity purification and microflow LC-ESI-Q-TOF Mass Spectrometry. J Am Soc Mass Spectrom. 2017;28(5):811-817. doi:10.1007/s13361-016-1566-y
10. Ladwig PM, Barnidge DR, Willrich MA. Mass spectrometry approaches for identification and quantitation of therapeutic monoclonal antibodies in the clinical laboratory. Clin Vaccine Immunol. 2017;24(5):e00545-16. doi:10.1128/CVI.00545-16
11. Kulasekararaj AG, Hill A, Rottinghaus ST, et al. Ravulizumab (ALXN1210) vs eculizumab in C5-inhibitor-experienced adult patients with PNH: the 302 study. Blood. 2019;133(6):540-549. doi:10.1182/blood-2018-09-876805
12. Stern RM, Connell NT. Ravulizumab: a novel C5 inhibitor for the treatment of paroxysmal nocturnal hemoglobinuria. Ther Adv Hematol. 2019;10:2040620719874728. doi:10.1177/2040620719874728
13. Alexion Pharmaceuticals. BLA 761108-S1 Multi-disciplinary review and evaluation: Ultomiris (ravulizumab-cwvz). FDA; April 2, 2019. Available at www.fda.gov/media/135113/download
14. Willrich MAV, Ladwig PM, Martinez MA, et al. Monitoring Ravulizumab effect on complement assays. J Immunol Methods. 2021;490:112944. doi:10.1016/j.jim.2020.112944
15. Wong EK, Goodship TH, Kavanagh D. Complement therapy in atypical haemolytic uraemic syndrome (aHUS). Mol Immunol. 2013;56(3):199-212
16. Rother RP, Rollins SA, Mojcik CF, Brodsky RA, Bell L. Discovery and development of the complement inhibitor eculizumab for the treatment of paroxysmal nocturnal hemoglobinuria. Nat Biotechnol. 2007;25(11):1256-1264
17. Zuber J, Le Quintrec M, Krid S, et al. Eculizumab for atypical hemolytic uremic syndrome recurrence in renal transplantation. Am J Transplant. 2012;12(12):3337-3354
18. Andreguetto B, Murray D, Snyder M, et al: The impact of eculizumab in complement assays. Mol Immunol. 2015;67:119-120
19. Willrich MAV, Braun KMP, Moyer AM, Jeffrey DH, Frazer-Abel A. Complement testing in the clinical laboratory. Crit Rev Clin Lab Sci. 2021;58(7):447-478. doi:10.1080/10408363.2021.1907297
20. Pittock SJ, Berthele A, Fujihara K, et al: Eculizumab in aquaporin-4-positive neuromyelitis optica spectrum disorder. N Engl J Med. 2019;381(7):614-625. doi:10.1056/NEJMoa1900866
21. Dhillon S. Eculizumab: A review in generalized myasthenia gravis. Drugs. 2018;78(3):367-376. doi:10.1007/s40265-018-0875-9
22. Howard JF Jr, Utsugisawa, K, Benatar M, et al. Safety and efficacy of eculizumab in anti-acetylcholine receptor antibody-positive refractory generalised myasthenia gravis (REGAIN): a phase 3, randomised, double-blind, placebo-controlled, multicentre study. Lancet Neurol. 2017;16(12):976-986. doi:10.1016/ S1474-4422(17)30369-1
Day(s) Performed
Varies
Report Available
3 to 12 daysPerforming Laboratory
Mayo Clinic Laboratories in Rochester
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.CPT Code Information
80299
86161
LOINC Code Information
| Test ID | Test Order Name | Order LOINC Value |
|---|---|---|
| ECMP | Eculizumab Monitoring Panel, S | 101922-3 |
| Result ID | Test Result Name | Result LOINC Value |
|---|---|---|
| 65676 | Eculizumab, S | 90240-3 |
| 619951 | Eculizumab Interpretation | 59462-2 |
| 618697 | Eculizumab Complement Blockage, S | 74520-8 |
Forms
If not ordering electronically, complete, print, and send a Coagulation Test Request (T753) with the specimen.