Description

Emapalumab-lzsg (Gamifant®) is an interferon gamma blocking antibody for the treatment of newborn and older individuals with primary hemophagocytic lymphohistiocytosis (HLH) with refractory, recurrent, or progressive disease or intolerance with conventional HLH therapy.

Criteria

Coverage is subject to the specific terms of the member’s benefit plan.

Federal Employee Program members (FEP) should check with their Retail Pharmacy Program to determine if prior approval is required by calling the Retail Pharmacy Program at 1-800-624-5060 (TTY: 1-800-624-5077). FEP members can also obtain the list through the www.fepblue.org website.

Emapalumab (Gamifant) may be considered medically necessary in individuals newborn and older when ALL of the following criteria are met:

• Submission of recent medical records confirming ONE of the following:
  • Confirmation of gene mutation known to cause primary HLH (i.e., PRF1, UNC13D, STX11, STXBP2, etc.); or
  • Confirmation individual meets FIVE of the following EIGHT criteria:
    • Fever (defined as 101.3 °F); or
    • Splenomegaly; or
    • Cytopenia affecting TWO of following:
      • Hemoglobin less than 9 g/dL; or
      • Platelets less than 100 x 109/L; or
      • Neutrophils less than 1 x 109/L; and
    • ONE of the following:
      • Hypertriglyceridemia (defined as fasting triglycerides greater than 3 mmol/L, greater than or equal to 265 mg/dL, or greater than 3 standard deviations of normal value for individual’s age); or
      • Hypofibrinogenemia (defined as less than or equal to 1.5 g/L); and
    • Hemophagocytosis in bone marrow, spleen, or lymph nodes with no evidence of malignancy; or
    • Low or absent natural killer cell activity; or
    • High ferritin (defined as equal to or greater than 500 mcg/L); or
    • High soluble CD25 (defined as equal to or greater than 2,400 U/mL); and
• Individual has ONE of the following with conventional HLH therapy (e.g., high dose corticosteroid, etoposide, cyclosporine A regimen):
  • Refractory, recurrent, or progressive disease; or
  • Intolerance, adverse effects, or contraindication to conventional HLH therapy; and
• Emapalumab (Gamifant) to be administered with dexamethasone, with dexamethasone-naïve individuals initiating dexamethasone the day before emapalumab (Gamifant) therapy; and
• Individual is a suitable candidate for stem cell transplantation (SCT); and
• Emapalumab (Gamifant) to be discontinued upon initiation of SCT and not readministered after SCT; and
• Individual will not receive live or live attenuated vaccinations during treatment or for four weeks after last dose of emapalumab (Gamifant).

 The use of emapalumab-lzsg (Gamifant) for any other indication is considered experimental/investigation and therefore non-covered. Scientific evidence does not support its use for any other indications.

Procedure Codes

Note: Emapalumab (Gamifant) is not indicated for the treatment of secondary HLH. 

Note: Prior to emapalumab (Gamifant) administration, individual should be evaluated for infection, including latent tuberculosis (TB). Prophylaxis for TB should be administered to individuals who are at risk for TB or known to have positive purified protein derivative (PPD) test result or positive interferon gamma release assay. Individuals should also be administered prophylaxis for herpes zoster, Pneumocystis jirovecii, and fungal infections prior to emapalumab (Gamifant) administration. During treatment, individuals should be monitored for TB, adenovirus, Epstein-Barr virus (EBV), and cytomegalovirus (CMV) every two weeks and as clinically indicated. 

Note: In addition to the above criteria, product specific dosage and/or frequency limits may apply in accordance with the U.S. Food and Drug Administration (FDA)-approved product prescribing information, national compendia, Centers for Medicare and Medicaid Services (CMS) and other peer reviewed resources or evidence-based guidelines. Blue Cross Blue Shield of North Dakota may deny, in full or in part, reimbursement for utilization that does not fall within the applicable dosage and/or frequency limits.

Diagnosis Codes

Links

References (PDF)