Criteria
Coverage is subject to the specific terms of the member’s benefit plan.
The use of emapalumab-lzsg (Gamifant) may be considered medically necessary when ALL of the following criteria are met:
- The individual must meet criteria as outlined in prescribing information (PI) including recommendations for diagnosis and age; and
- The prescriber must be, or in consultation with a hematologist, oncologist, immunologist, or transplant specialist; and
- The individual must have diagnosis of primary hemophagocytic lymphohistiocytosis (HLH); and
- The individual has refractory, recurrent or progressive disease or intolerance with conventional HLH therapy (i.e., etoposide + dexamethasone, cyclosporine A, or Anti-thymocyte globulin); and
- The individual must be a candidate for stem cell transplant; and
- The individual must have one of the following:
- Confirmation of a gene mutation known to cause primary HLH (e.g. PRF1, UNC13D, STX11 RAB27A, STXBP2); or
- Confirmation of 5 of the following clinical characteristics:
- Fever ≥3F for over 7 days
- Splenomegaly
- Two of the following cytopenias in the peripheral blood:
- Hemoglobin < 9 g/dL (or < 10 g/dL in infants less than 4 weeks of age)
- Platelet count < 100,000/microL
- ANC <1000/microL
- One of the following:
- Hypertriglyceridemia defined as fasting triglycerides ≥ 265 mg/dL (2 mmol/L)
- Hypofibrinogenemia defined as fibrinogen ≤5 g/L
- Hemophagocytosis in bone marrow or spleen or lymph nodes with no evidence of malignancy
- Low or absent natural killer cell activity
- Ferritin ≥ 500 mg/L
- Soluble CD25 (i.e., soluble IL-2 receptor) ≥ 2,400 U/mL; and
- Emapalumab-lzsg (Gamifant) must be administered with dexamethasone as part of the induction or maintenance phase of stem cell transplant, which is to be discontinued at the initiation of conditioning for stem cell transplant.
Initial Authorization: 3 months or up to the hematopoietic stem cell transplantation (HSCT) date
Reauthorization Criteria
Continuation of therapy with emapalumab-lzsg (Gamifant) may be considered medically necessary when the following is met:
- At least 3 HLH abnormalities must be improved by at least 50% from baseline.
Continuation Authorization: 3 months or up to the hematopoietic stem cell transplantation (HSCT) date
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
