CurrentCareSourcePolicy N/A

UTILIZATION MANAGEMENT MEDICAL POLICY

Policy Summary

PayerCareSource

PolicyUTILIZATION MANAGEMENT MEDICAL POLICY

Policy CodePolicy N/A

Change TypeAnnual Revision (no criteria changes)

Effective Date

Next Review Date

Key ActionPrior Authorization is recommended; approve only patients meeting the policy criteria and dosing, with approvals provided for 1 year.

SourceLink

POLICY UPDATE CHANGES

Annual Revision, Review Date = 01/22/2025 with no criteria changes.

1Covered Indication (cold agglutinin disease)

8Required clinical criteria items

1Approval duration (years)

Coverage Summary

Policy governs medical-benefit coverage and prior authorization criteria for Enjaymo (sutimlimab-jome) intravenous infusion for treatment of hemolysis in adults with cold agglutinin disease. Coverage stance: covered_with_criteria. Approval duration is 1 year. This policy establishes diagnostic, baseline, dosing, prescriber specialty, and exclusion requirements and is used to manage medical-benefit prior authorization requests for Enjaymo.

Initial Therapy Criteria

FDA-Approved Indication — Cold Agglutinin Disease (approve for 1 year)

Approve for 1 year if the patient meets ALL of the following (A, B, C, D, E, F, G, and H):

ALL of the following

Patient is ≥ 18 years of age
Patient weighs ≥ 39 kg≥ 39 kg
Patient has a history of at least one sign or symptom associated with cold agglutinin disease
Examples include symptomatic anemia (fatigue, weakness, shortness of breath, palpitations, lightheadedness, chest pain), acrocyanosis, Raynaud's syndrome, hemoglobinuria, disabling circulatory symptoms, or a major adverse vascular event
According to the prescriber, the patient has evidence of chronic hemolysis
DAT and Ig findings
- Direct antibody test strongly positive for C3d
- Direct antibody test negative or only weakly positive for immunoglobulin G
Diagnostic criteria (both required)
- Hemoglobin < 10 g/dL< 10 g/dL
- Cold agglutinin antibody titer ≥ 64 at 4°C (approximately 40 Hemagglutination titer PF)≥ 64 at 4°C
Baseline lab and exclusion (both required)
- Total bilirubin above the upper limit of normal (based on reporting laboratory reference range) prior to initiationAbove upper limit of normal
- According to the prescriber, secondary causes of cold agglutinin syndrome have been excluded
  Examples of secondary causes include infection, rheumatologic diseases, and active hematologic malignancies
Enjaymo is prescribed by or in consultation with a hematologist

Dosing and Administration (Approved Regimens)

Dosing — approved regimens

Approve the following dosing regimens:

ANY of the following

For a patient weighing 39 to <75 kg: recommended dose is 6,500 mg6,500 mg
Initial dosing once weekly for 2 weeks, then every 2 weeks (Q2W). If interval between doses exceeds 17 days, re-initiate once weekly for 2 weeks then return to Q2W.
For a patient weighing ≥75 kg: recommended dose is 7,500 mg7,500 mg
Initial dosing once weekly for 2 weeks, then every 2 weeks (Q2W). If interval between doses exceeds 17 days, re-initiate once weekly for 2 weeks then return to Q2W.

Billing Rule

Dosing outside policy

Requests for doses outside of the dosing documented in this policy will be reviewed on a case-by-case basis by a clinician (Medical Director or Pharmacist).

Provider Actions & Documentation Requirements

Prior Authorization

Prior authorization required

Prior authorization is recommended for medical benefit coverage of Enjaymo. Approval is recommended only for patients who meet the policy criteria and approved dosing. All approvals are provided for a duration of 1 year.

Documentation Required

Diagnostic and baseline documentation required

Providers must document baseline diagnostic and laboratory evidence to support use, including the items listed below.

Evidence of chronic hemolysis (as documented by the prescriber)
Direct antiglobulin test (DAT) results: strongly positive for C3d and negative or only weakly positive for IgG
Cold agglutinin antibody titer ≥ 64 at 4°C
Hemoglobin < 10 g/dL
Total bilirubin above the upper limit of normal (based on reporting laboratory reference range) prior to initiation
Exclusion of secondary causes of cold agglutinin syndrome (e.g., infection, rheumatologic disease, active hematologic malignancy)
Relevant signs or symptoms associated with cold agglutinin disease (examples: symptomatic anemia, acrocyanosis, Raynaud’s phenomena, hemoglobinuria, disabling circulatory symptoms)

Documentation Required

Specialist prescribing requirement

Enjaymo must be prescribed by or in consultation with a hematologist.

Clinical Evidence

24CARDINAL trial participants (required recent transfusion)

42CADENZA trial participants (no recent transfusion required)

supportiveSupportive efficacy data cited (additional Phase III data and long-term studies referenced)

Enjaymo is a classical complement inhibitor indicated for treatment of hemolysis in cold agglutinin disease in adults. Key Phase III trials include CARDINAL (n=24), which required recent transfusion and enrolled patients with confirmed chronic hemolysis and typical DAT findings, and CADENZA (n=42), which required chronic hemolysis and diagnostic criteria but did not require recent transfusion. Dosing is weight-based with initial once-weekly dosing for 2 weeks then every 2 weeks thereafter.

Background & Definitions

Cold agglutinin disease is a rare autoimmune hemolytic anemia characterized by chronic hemolysis, a cold agglutinin titer ≥64 at 4°C, and typical direct antiglobulin test (DAT) findings of strong C3d positivity with IgG negative or only weakly positive. Patients may have symptomatic anemia and circulatory symptoms (ranging from acrocyanosis to severe Raynaud phenomena). Secondary causes (cold agglutinin syndrome) such as malignancy, infection, and autoimmune diseases must be excluded before diagnosing primary cold agglutinin disease.

Cold agglutinin disease: Primary autoimmune hemolytic anemia defined by chronic hemolysis, cold agglutinin titer ≥64 at 4°C, DAT strongly positive for C3d and IgG negative/weak; secondary causes (infection, rheumatologic disease, hematologic malignancy) excluded.

Exclusions / Unproven Uses

Exclusions: secondary causes of cold agglutinin syndrome must be excluded (examples include infection, rheumatologic diseases, and active hematologic malignancies). Policy applies to adult patients (age ≥18 years) and governs medical-benefit use only; Enjaymo should be prescribed by or in consultation with a hematologist. The brief lists no additional unproven indications for Enjaymo; coverage is limited to the recommended indication and criteria.