CurrentUnitedHealthcarePolicy CSIND0077.07

Medical Benefit Drug Policy

UnitedHealthcare Medical Benefit Drug Policy CSIND0077.07 governs medical necessity criteria, duration, and billing codes for Gamifant (emapalumab-lzsg) for members of the Indiana Community Plan. It specifies initial and continuation criteria for primary HLH and HLH/MAS in Still's disease, exclusions for secondary HLH, administration constraints, and applicable HCPCS and ICD-10 codes.

Policy Summary

PayerUnitedHealthcare

PolicyMedical Benefit Drug Policy

Policy CodePolicy CSIND0077.07

Change TypeRevised coverage criteria

Effective DateJan 1, 2026

Next Review Date

Key ActionObtain prior authorization with submission of medical records confirming diagnostic criteria, prior therapy history, HSCT candidacy, concomitant dexamethasone use, and dosing per FDA labeling.

POLICY UPDATE CHANGES

Revised coverage criteria; added criterion requiring the provider does not request a planned inpatient admission for the sole purpose of administering Gamifant.

Replaced references to 'emapalumab' with 'Gamifant'.

2Covered Indications (primary HLH; HLH/MAS in Still's disease)

1Not Covered Indications (secondary HLH)

6-12 monthsAuthorization Durations (primary HLH / HLH-MAS)

Coverage Summary & Indications

UnitedHealthcare Medical Benefit Drug Policy CSIND0077.07 (Gamifant; emapalumab-lzsg) applies to Indiana Community Plan members and defines medical necessity criteria, duration limits, and applicable billing codes for Gamifant. The policy is effective 2026-01-01 and is identified as Medical Benefit Drug Policy CSIND0077.07 (UnitedHealthcare). The policy governs coverage for primary HLH and HLH/MAS in Still's disease, specifies authorization durations and prior authorization requirements, and lists relevant HCPCS and ICD-10 codes (e.g., J9210).

HLH/MAS in Still's disease (sJIA or AOSD) - Initial Therapy (Medically Necessary)

Gamifant is medically necessary for HLH/MAS in Still's disease when ALL of the following initial therapy criteria are met:

ALL of the following

Documentation
- Diagnosis: Confirmed or suspected diagnosis of systemic juvenile idiopathic arthritis (sJIA) or adult onset Still's disease (AOSD)
- Active MAS diagnosis
  - Ferritin > 684 ng/mL> 684 ng/mL
  - Two of the following laboratory criteria
    Platelets: Platelet count ≤ 181 x 10^9 /L≤ 181 x 10^9 /L
    AST > 48 U/L> 48 U/L
    Triglycerides > 156 mg/dL> 156 mg/dL
    Fibrinogen level ≤ 360 mg/dL≤ 360 mg/dL
Additional initial requirements
- Prior steroid failure: Patient has had an inadequate response to high-dose intravenous glucocorticoids
- Admission restriction: Provider does not request a planned inpatient admission for the sole purpose of administering Gamifant
- FDA dosing: Dosing is in accordance with the United States FDA approved labeling
- Initial duration: Initial authorization will be for no more than 12 months≤ 12 months

Continuation / Reauthorization Criteria

HLH/MAS in Still's disease - Continuation of Therapy (Medically Necessary)

Reauthorization requires ALL of the following:

Continuation requirements

Clinical response: Documentation of a positive clinical response to Gamifant
Admission restriction: Provider does not request a planned inpatient admission for the sole purpose of administering Gamifant
FDA dosing: Dosing is in accordance with the FDA approved labeling
Reauthorization duration: Reauthorization will be for no more than 12 months≤ 12 months

Unproven / Not Medically Necessary

Not Medically Necessary / Not Proven

Gamifant is not proven or medically necessary for the following:

Exclusions

Secondary HLH: Treatment of secondary HLH

Prior Authorization, Documentation & Billing Rules

Prior Authorization

Prior authorization required

Authorization is required. Submit medical records confirming genetic testing or fulfillment of the clinical HLH criteria, prior treatment history (inadequate response or intolerance to standard therapy), that the patient is a candidate for HSCT, concomitant administration with dexamethasone, and dosing per the FDA label. Authorizations are limited to no more than 6 months for primary HLH; initial and reauthorization for HLH/MAS are limited to no more than 12 months.

J9210

Documentation Required

Clinical documentation to support diagnosis

Submit chart notes and laboratory values that confirm either a causative genetic mutation for primary HLH or fulfillment of five diagnostic HLH criteria (e.g., fever, splenomegaly, cytopenias, hypertriglyceridemia or hypofibrinogenemia, hemophagocytosis, low/absent NK activity, ferritin ≥ 500 mcg/L, soluble CD25 ≥ 2,400 U/mL). For MAS in sJIA/AOSD, submit documentation of ferritin > 684 ng/mL plus two of the listed lab criteria. Also document prior therapy failure or intolerance and, for continuation/reauthorization, evidence of a positive clinical response to Gamifant.

Ferritin ≥ 500 mcg/L
Soluble CD25 ≥ 2,400 U/mL
Hypertriglyceridemia: fasting triglycerides ≥ 3 mmol/L or ≥ 265 mg/dL
Hypofibrinogenemia (primary HLH): fibrinogen ≤ 1.5 g/L
MAS ferritin: > 684 ng/mL
MAS platelets: ≤ 181 x 10^9 /L
MAS AST: > 48 U/L
MAS triglycerides: > 156 mg/dL
MAS fibrinogen: ≤ 360 mg/dL

Billing Rule

Administration site / admission restriction

The provider must not request a planned inpatient admission solely for the purpose of administering Gamifant; such admission-based requests may be denied under the revised criteria effective 2026-01-01.

J9210 — requests for admission-based administration risk denial

Documentation Required

HSCT timing

When Gamifant is used as part of induction or maintenance for HSCT, therapy must be discontinued at initiation of conditioning for transplant and the patient must be a candidate for HSCT. Documentation should map to the authorization requirements (e.g., chart notes confirming HSCT candidacy and planned conditioning timing).

Clinical Evidence Summary

53.8%Complete response (pooled HLH/MAS studies); 95% CI: 37.2–69.9

Clinical evidence includes the phase 2–3 NI-0501-04 trial in primary HLH, which reported an overall response of 64.7% at end of treatment, an 88.2% response rate overall, and a median time to first response of 8 days, with many patients bridged to hematopoietic stem cell transplantation. For HLH/MAS in Still's disease, pooled open-label studies (NI-0501-06 and NI-0501-14) reported a composite complete response in 53.8% (95% CI: 37.2, 69.9). Key safety concerns observed across the NI-0501 studies included frequent infections (bacterial, fungal, viral, including CMV), infusion-related reactions, and serious adverse events such as aggravated HLH and respiratory failure in the pre-conditioning period, and transplant-related complications (e.g., graft failure, sepsis) in the post-conditioning period.

Background & Definitions

Background: Gamifant (emapalumab-lzsg) is an interferon gamma (IFNγ) neutralizing monoclonal antibody; nonclinical data indicate IFNγ is hypersecreted in HLH and emapalumab reduces IFNγ-driven biomarkers (e.g., CXCL9). It is FDA-indicated for primary HLH with refractory, recurrent, or progressive disease or intolerance to conventional therapy, and for HLH/MAS in known or suspected Still's disease (including sJIA) with inadequate response or intolerance to glucocorticoids. Clinical trials demonstrated the ability to achieve disease control and bridge patients to hematopoietic stem cell transplantation (NI-0501-04 showed 64.7% overall response and many patients proceeded to HSCT), and pooled HLH/MAS studies showed a 53.8% complete response

Term	Definition
MAS	Macrophage activation syndrome, a form of HLH occurring in rheumatic diseases such as Still's disease.
HSCT	Hematopoietic stem cell transplant.

Key Numeric Thresholds

Laboratory thresholds for primary HLH and MAS in Still's disease

Ferritin (primary HLH)≥ 500 mcg/L

Soluble CD25 (soluble IL-2 receptor)≥ 2,400 U/mL

Hypertriglyceridemia (primary HLH)Fasting triglycerides ≥ 3 mmol/L or ≥ 265 mg/dL

Hypofibrinogenemia (primary HLH)Fibrinogen ≤ 1.5 g/L

MAS ferritin (sJIA/AOSD)> 684 ng/mL

MAS platelets (sJIA/AOSD)≤ 181 x10^9/L

MAS AST (sJIA/AOSD)> 48 U/L

MAS triglycerides (sJIA/AOSD)> 156 mg/dL

MAS fibrinogen (sJIA/AOSD)≤ 360 mg/dL

Policy Revision History

2026-01-01material_change_effectiveLatest

Revised coverage criteria effective 2026-01-01: criteria updated to include a requirement that the provider does not request a planned inpatient admission for the sole purpose of administering Gamifant; authorization durations and other clinical criteria retained/updated as specified in policy CSIND0077.07.

2026-01-01non_material_text_change

Non-material textual change: replaced references to 'emapalumab' with 'Gamifant' throughout the policy (archived prior version CSIND0077.06).

Policy Summary

PayerUnitedHealthcare

PolicyMedical Benefit Drug Policy

Policy CodePolicy CSIND0077.07

Change TypeRevised coverage criteria

Effective DateJan 1, 2026

Next Review Date

On This Page

D76.1	Hemophagocytic lymphohistiocytosis
D76.2	Hemophagocytic syndrome, infection-associated
D76.3	Other histiocytosis syndromes

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