CurrentAvMedPolicy N/A

Gamifant – Primary Hemophagocytic Lymphohistiocytosis (HLH) (Medical)

Prior authorization and step-edit policy for medical benefit use of Gamifant (J9210) to treat primary HLH, defining initial and reauthorization clinical criteria, dosing escalation rules, safety screening/monitoring requirements, administration site/fill options, and documentation/submission instructions.

Policy Summary

PayerAvMed

PolicyGamifant – Primary Hemophagocytic Lymphohistiocytosis (HLH) (Medical)

Policy CodePolicy N/A

Change TypeNo material change

Effective Date

Next Review Date

Key ActionFax the signed prior authorization form with supporting documentation (labs, genetics, chart notes) to 1-877-535-1391; incomplete or illegible information may delay or deny authorization.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes

1Primary HCPCS codes listed

6 monthsAuthorization duration (initial & reauthorization)

5 of 8Diagnostic criteria threshold

Coverage Summary

Coverage stance: covered_with_criteria for Gamifant (emapalumab-lzsg) when used for primary HLH under the medical benefit (HCPCS/J-code J9210). Prior authorization is required. Initial authorization and reauthorization durations are each 6 months. Gamifant is intended for use as part of induction/maintenance prior to hematopoietic stem cell transplant (HSCT) and will be discontinued at the initiation of conditioning for HSCT.

Initial Authorization (6 months) - Medical Necessity Criteria

All criteria must be met for approval. To support each line checked, all documentation, including lab results, diagnostics, and/or chart notes, must be provided or request may be denied.

ALL of the following

Prescriber specialty requirement: Medication is prescribed by or in consultation with a hematologist, oncologist, immunologist, transplant specialist, or physician who specializes in hemophagocytic lymphohistiocytosis
Definitive diagnosis of HLH by ONE of the following
- Genetic or family history diagnosis: A diagnosis of primary HLH based on identification of biallelic pathogenic gene variants from molecular genetic testing (e.g., PRF1, UNC13D, STX11, or STXBP2) or a family history consistent with primary HLH
  submit documentation
- At least FIVE of the following eight documented criteria
  - Fever criterion: Prolonged fever (≥101.3 °F) > 7 days≥101.3 °F for > 7 days
  - Splenomegaly
  - Cytopenias affecting 2 of 3 lineages in the peripheral blood (hemoglobin < 9 g/dL, platelets < 100 x 10^9/L, neutrophils < 1 x 10^9/L)Hgb < 9 g/dL; platelets < 100 x10^9/L; neutrophils < 1 x10^9/L
  - Triglycerides / fibrinogen: Hypertriglyceridemia (fasting triglycerides > 3 mmol/L or ≥ 265 mg/dL) and/or hypofibrinogenemia (≤ 1.5 g/L)>3 mmol/L or ≥265 mg/dL; ≤1.5 g/L
Refractory/recurrent disease requirement: Member has active, primary disease that is refractory, recurrent, or progressive during treatment with conventional HLH therapy (e.g., etoposide + dexamethasone, cyclosporine A, anti-thymocyte globulin, etc.) unless patient is intolerant to conventional HLH therapy
HSCT status: Member has NOT received hematopoietic stem cell transplant (HSCT)
NOTE: Gamifant is being used as part of the induction or maintenance phase of stem cell transplant, and will be discontinued at the initiation of conditioning for stem cell transplant
TB screening and monitoring: Member has been evaluated and screened for the presence of latent tuberculosis (TB) infection prior to initiating treatment and will receive ongoing monitoring for the presence of TB during treatment
Infection prophylaxis monitoring: Provider will monitor and consider prophylaxis in patients for Herpes Zoster, Pneumocystis Jirovecii, and fungal infections
Absence of active infection: Member does NOT have an active infection, including clinically important localized infections that are favored by interferon-gamma neutralization (e.g., infections caused by mycobacteria, Histoplasma Capsulatum, etc.)
No live vaccines concurrent: Provider attests that the member will NOT be administered concurrently with live or live attenuated vaccines
Combination with dexamethasone and continuation allowance: Requested medication will be used in combination with dexamethasone
NOTE: Patients currently on oral cyclosporine A, or intrathecal methotrexate and/or glucocorticoids may continue on therapy while treated with emapalumab

Reauthorization (6 months) - Continued Therapy Criteria

All criteria must be met for approval. To support each line checked, all documentation, including lab results, diagnostics, and/or chart notes, must be provided or request may be denied.

ALL of the following

Concomitant therapy requirement: Member continues to meet criteria such as concomitant therapy requirements (not including prerequisite therapy) identified in the initial criteria section above
Ongoing need: Member continues to require therapy for treatment of HLH (e.g., until HSCT is performed or unacceptable toxicity)
Evidence of disease improvement defined by ONE of the following
- Complete response defined as normalization of all HLH abnormalities (i.e., no fever, no splenomegaly, neutrophils > 1x10^9/L, platelets > 100x10^9/L, ferritin < 2,000 μg/L, fibrinogen > 1.50 g/L, D-dimer < 500 μg/L, normal CNS symptoms, no worsening of sCD25 > 2-fold baseline)ferritin < 2000 μg/L; D-dimer < 500 μg/L; neutrophils >1x10^9/L; platelets >100x10^9/L; fibrinogen >1.50 g/L
- Partial response defined as normalization of ≥ 3 HLH abnormalities (including CNS abnormalities)normalization of ≥3 abnormalities
- HLH improvement defined as improvement by at least 50% from baseline of ≥ 3 HLH clinical and laboratory criteria (see initial criteria section)≥50% improvement from baseline of ≥3 criteria
Combination with dexamethasone: Requested medication will be used in combination with dexamethasone
NOTE: Patients currently on oral cyclosporine A, or intrathecal methotrexate and/or glucocorticoids may continue on therapy while treated with emapalumab
Absence of unacceptable toxicity: Member has experienced an absence of unacceptable toxicity from the drug (e.g., serious infections (including mycobacteria, Herpes Zoster virus, and Histoplasma Capsulatum), infusion-related reactions (including drug eruption, pyrexia, rash, erythema, and hyperhidrosis))

Dose Escalation Criteria (Days 1–10) & Administration Notes

Dose Escalation Criteria (Days 1–10)

Dose increases allowed up to specified maximums when unsatisfactory improvement is documented; criteria reference clinical and laboratory parameters.

Administration context (operational note): Gamifant is used as part of induction or maintenance therapy prior to HSCT and will be discontinued at the initiation of conditioning for HSCT. Dosing is weight-based and escalates on Days 1, 4, 7, and 10 (starting dose 1 mg/kg on Day 1, with planned increases to 3 mg/kg, 6 mg/kg, and 10 mg/kg per the escalation schedule).

Provider Actions & Submission Requirements

Prior Authorization

Submit prior authorization request

Prescribing physician must sign and clearly print name on the prior authorization request form (preprinted stamps not valid). Fax the completed form with supporting documentation (labs, genetics, chart notes) to 1-877-535-1391. If information provided is not complete, correct, or legible, authorization can be delayed or denied.

Documentation Required

Provide diagnostic and laboratory evidence

Submit documentation supporting a definitive HLH diagnosis (molecular genetic testing showing biallelic pathogenic variants or documentation of at least 5 of the 8 HLH criteria), baseline laboratory results, and ongoing monitoring results. Failure to provide required documentation may result in denial.

Documentation Required

TB screening and infection monitoring

Evaluate and screen for latent tuberculosis (TB) infection prior to initiating treatment and provide ongoing monitoring for TB during treatment. Provider should monitor and consider prophylaxis for Herpes Zoster, Pneumocystis jirovecii, and fungal infections while on therapy.

Documentation Required

HSCT status attestation

Provider must attest on the request form that the member has not received hematopoietic stem cell transplant (HSCT). Note: Gamifant will be discontinued at the initiation of conditioning for HSCT.

Billing Rule

Administration location or specialty pharmacy selection

Indicate whether the medication will be provided at a specified administration location (include NPI or DEA number of the administering location) or via a specialty pharmacy on the form.

Documentation Required

No concurrent live vaccines

Provider must attest that the member will NOT be administered live or live-attenuated vaccines while receiving therapy.

Billing Rule

Use of samples

Use of samples to initiate therapy does not meet step edit/preauthorization criteria and therefore does not satisfy prior authorization requirements.

Denial Risk

Urgent review criteria

For urgent review requests, the practitioner should call the AvMed Pre-Authorization Department. AvMed defines urgent as situations where lack of treatment could seriously jeopardize the life or health of the member or their ability to regain maximum function; standard review timelines may be insufficient in these cases.

Safety Thresholds & Definitions

Clinical & laboratory thresholds

Fever for HLH criterion≥101.3 °F for > 7 days

Hemoglobin threshold (cytopenia)< 9 g/dL

Platelet threshold (cytopenia)< 100 x 10^9/L

Neutrophil threshold (cytopenry)< 1 x 10^9/L

Hypertriglyceridemia> 3 mmol/L or ≥ 265 mg/dL

Hypofibrinogenemia≤ 1.5 g/L

Ferritin diagnostic≥ 500 mcg/L

Soluble CD25≥ U/mL (unit unspecified)

Reauthorization ferritin response thresholdFerritin < 2,000 μg/L (complete response)

Dose escalation ferritin triggerEither baseline ≥ 3,000 ng/mL with <20% decrease OR baseline < 3,000 ng/mL with increase to > 3,000 ng/mL

HLH: Hemophagocytic lymphohistiocytosis — a hyperinflammatory disorder defined by genetic or clinical criteria.

HSCT: Hematopoietic stem cell transplant — definitive cellular therapy for primary HLH; Gamifant is used prior to transplant and discontinued at conditioning.

sCD25: Soluble CD25 (soluble IL-2Rα receptor) — a laboratory marker included among HLH diagnostic criteria.

Policy Summary

PayerAvMed

PolicyGamifant – Primary Hemophagocytic Lymphohistiocytosis (HLH) (Medical)

Policy CodePolicy N/A

Change TypeNo material change

Effective Date

Next Review Date

Key ActionFax the signed prior authorization form with supporting documentation (labs, genetics, chart notes) to 1-877-535-1391; incomplete or illegible information may delay or deny authorization.

SourceLink

On This Page

OpenPayer

Gamifant – Primary Hemophagocytic Lymphohistiocytosis (HLH) (Medical)

Coverage Summary

Initial Authorization (6 months) - Medical Necessity Criteria

Reauthorization (6 months) - Continued Therapy Criteria

Dose Escalation Criteria (Days 1–10) & Administration Notes

Provider Actions & Submission Requirements

Applicable Codes

Safety Thresholds & Definitions

Background and Clinical Context