ENJAYMO (sutimlimab-jome)

ENJAYMO (sutimlimab-jome)

Medical Administration – intravenous infusion

Diagnosis considered for coverage:

• Cold Agglutinin Disease (CAD) - Indicated to decrease the need for red blood cell (RBC) transfusion due to hemolysis in adults with cold agglutinin disease.

Coverage Criteria:

For diagnosis of Cold Agglutinin Disease (CAD)

• Dose does not exceed one of the following:
  • Patients weighing between 39 kg to less than 75 kg: 6,500 mg on day 0, 7, and every 14 days thereafter 
  • Patients weighing 75 kg or greater: 7,500 mg on day 0, 7, and every 14 days thereafter, AND
• Patient weighs at least 39 kg, AND
• Patient is 18 years of age or older, AND
• Prescribed by or in consultation with a hematologist, AND
• Diagnosis of CAD is based on ALL of the following:
  • Presence of chronic hemolysis (e.g. bilirubin level above the normal reference range, elevated lactated dehydrogenase [LDH], decreased haptoglobin, increased reticulocyte count) 
  • Positive polyspecific direct antiglobulin test (DAT) 
  • Monospecific DAT strongly positive for C3d
  • Cold agglutinin titer greater than or equal to 64 measured at 4 degree Celsius 
  • Direct antiglobulin test (DAT) result for Immunoglobulin G (IgG) of 1+ or less; AND
• Baseline hemoglobin level less than or equal to 10.0 gram per deciliter (g/dL); AND
• Patient does not have cold agglutinin syndrome secondary to other factors (e.g., overt hematologic malignancy, infection, rheumatologic disease, systemic lupus erythematosus or other autoimmune disorders); AND
• One of the following (1 OR 2):
  • 1. Patient has had one prior blood transfusion in the prior 6 months AND has tried and failed or is not a candidate for rituximab-based regimens OR 
  • 2. All of the following (a, b and c):
    • a. Enjaymo is being used for short duration (e.g., prior to cardiac surgery to prevent exacerbations) or as a treatment bridge to rituximab-based regimens, and
    • b. Patient has need for rapidly acting treatment, and
    • c.  Patient has contraindication(s) to blood transfusions

Reauthorization Criteria:

For diagnosis of Cold Agglutinin Disease (CAD):

• Dose does not exceed one of the following:
  • Patients weighing between 39 kg to less than 75 kg: 6,500 mg on day 0, 7, and every 14 days thereafter
  • Patients weighing 75 kg or greater: 7,500 mg on day 0, 7, and every 14 days thereafter, AND
• Prescribed by or in consultation with a hematologist, AND
• Documentation of a positive clinical response to therapy as evidenced by ALL of the following:
  • The patient has not required any blood transfusions after the first 5 weeks of therapy with Enjaymo 
  • Hemoglobin level greater than or equal to 12 gram per deciliter (g/dL) or increased greater than or equal to 2 g/dL from baseline
     

Coverage Duration: 

• Initial: 6 months
• Reauthorization: 12 months

Authorization is not covered for the following:

The use of this drug for indications not listed in this policy does not meet the coverage criteria established by the Western Health Advantage (WHA) Pharmacy and Therapeutics (P&T) Committee.

Additional Information: 

• Weight-Based Dosing:

The dosing is 6,500 mg or 7,500 mg Enjaymo (based on body weight) intravenously over approximately 60 minutes on Day 0, Day 7, and every 14 days thereafter

• Enjaymo may increase susceptibility to serious infections, including infections caused by encapsulated bacteria such as Neisseria meningitides (any serogroup), Streptococcus pneumoniae, and Haemophilus influenzae. Vaccinate patients against encapsulated bacteria according to the most current ACIP recommendations.

Policy Updates:

• 08/16/2022 – New policy approved by P&T.

References:

1.    Enjaymo Prescribing Information. Bioverativ USA Inc. Waltham, MA. February 2022.
2.    Diagnosing Cold Agglutinin Disease (CAD) available at https://www.understandingcad.com/diagnosing-cold-agglutinin-disease/. Accessed March 8, 2022.
3.    A Study to Assess the Efficacy and Safety of BIVV009 (Sutimlimab) in Participants with Primary Cold Agglutinin Disease Who Have a Recent History of Blood Transfusion (Cardinal Study). Available at https://clinicaltrials.gov/ct2/show/NCT03347396. Accessed March 8, 2022.
4.    Roth, A., Barcellini, W., et al. Sutimlimab in Cold Agglutinin Disease. N Engl J Med 2021; 384:1323-1334. Available at https://www.nejm.org/doi/10.1056/NEJMoa2027760?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub%20%200pubmed. Accessed March 8, 2022
5.    Jäger U, Barcellini W, Broome CM, et al. Diagnosis and treatment of autoimmune hemolytic anemia in adults: Recommendations from the First International Congress Meeting. Blood Rev. 2020;41:100648.
6.    Brugnara, Carlo, and Sigbjorn Berentsen. “Cold Agglutinin Disease.” UpToDate. Accessed June 8, 2022. https://www.uptodate.com/contents/cold-agglutinin-disease.
7.    Alexander Röth, Wilma Barcellini, Shirley D'Sa, Yoshitaka Miyakawa, Catherine M Broome, Marc Michel, David J. Kuter, Bernd Jilma, Tor Henrik Anderson Tvedt, Ilene C. Weitz, Parija Patel, Xiaoyu Jiang, Caroline Reuter, Jun Su, Frank Shafer, Michelle Lee, William Hobbs, Sigbjørn Berentsen; Inhibition of Complement C1s with Sutimlimab in Patients with Cold Agglutinin Disease (CAD): Interim Results of the Phase 3 Cardinal Study Long-Term Follow-up. Blood 2020; 136 (Supplement 1): 24–25. doi: https://doi.org/10.1182/blood-2020-138909