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INDICATIONS

Gamifant (emapalumab-lzsg) is an interferon gamma (IFNγ)-neutralizing antibody indicated for the treatment of adult and pediatric (newborn and older) patients with:

  • Primary hemophagocytic lymphohistiocytosis (HLH) with refractory, recurrent, or progressive disease or intolerance with conventional HLH therapy.
  • HLH/macrophage activation syndrome (MAS) in known or suspected Still’s disease, including systemic Juvenile Idiopathic Arthritis (sJIA), with an inadequate response or intolerance to glucocorticoids, or with recurrent MAS.
IMPORTANT SAFETY INFORMATION
Infections

Gamifant may increase the risk of fatal and serious infections with pathogens including mycobacteria, herpes zoster virus... and histoplasma capsulatum. Do not administer Gamifant in patients with these infections until appropriate treatment has been initiated.

INDICATIONS

Gamifant (emapalumab-lzsg) is an interferon gamma (IFNγ)-neutralizing antibody indicated for the treatment of adult and pediatric (newborn and older) patients with:

  • Primary hemophagocytic lymphohistiocytosis (HLH) with refractory, recurrent, or progressive disease or intolerance with conventional HLH therapy.
  • HLH/macrophage activation syndrome (MAS) in known or suspected Still’s disease, including systemic Juvenile Idiopathic Arthritis (sJIA), with an inadequate response or intolerance to glucocorticoids, or with recurrent MAS.

IMPORTANT SAFETY INFORMATION

Infections

Gamifant may increase the risk of fatal and serious infections with pathogens including mycobacteria, herpes zoster virus, and histoplasma capsulatum. Do not administer Gamifant in patients with these infections until appropriate treatment has been initiated.

In patients with primary HLH receiving Gamifant in clinical trials, serious infections such as sepsis, pneumonia, bacteremia, disseminated histoplasmosis, necrotizing fasciitis, viral infections, and perforated appendicitis were observed in 32% of patients.

In patients with HLH/MAS in Still’s disease receiving Gamifant in clinical trials, serious infections such as pneumonia, cytomegalovirus infection, cytomegalovirus infection reactivation, and sepsis were observed in 13% of patients.

Evaluate patients for tuberculosis risk factors and test for latent infection prior to initiating Gamifant. Administer tuberculosis prophylaxis to patients at risk for tuberculosis or known to have a positive purified protein derivative (PPD) test result.

Consider prophylaxis for herpes zoster, Pneumocystis jirovecii, and fungal infection while receiving Gamifant. Employ surveillance testing during treatment with Gamifant.

Closely monitor patients receiving Gamifant for signs or symptoms of infection, promptly initiate a complete diagnostic workup appropriate for an immunocompromised patient, and initiate appropriate antimicrobial therapy.

Increased Risk of Infection With Use of Live Vaccines

Do not administer live or live attenuated vaccines to patients receiving Gamifant and for at least 4 weeks after the last dose of Gamifant. The safety of immunization with live vaccines during or following Gamifant therapy has not been studied.

Infusion-Related Reactions

Infusion-related reactions in patients with primary HLH, including drug eruption, pyrexia, rash, erythema, and hyperhidrosis, were reported with Gamifant treatment in 27% of patients. In one-third of these patients, the infusion-related reaction occurred during the first infusion.

Infusion-related reactions in patients with HLH/MAS in Still’s disease, including pyrexia, headache, paresthesia, bone pain, pruritic rash, and peripheral coldness, were reported with Gamifant treatment in 13% of patients. Infusion-related reactions were reported as mild in 8% of patients and as moderate in 5% of patients.

Monitor patients for infusion-related reactions, which can be severe. Interrupt the infusion for infusion reactions and institute appropriate medical management before continuing infusion at a slower rate.

Adverse Reactions

Primary HLH

Serious adverse reactions were reported in 53% of patients. The most common serious adverse reactions (≥3%) included infections, gastrointestinal hemorrhage, and multiple organ dysfunction. Fatal adverse reactions occurred in 2 (6%) of patients and included septic shock and gastrointestinal hemorrhage.

The most common adverse reactions were (≥10%) for Gamifant included infection (56%), hypertension (41%), infusion-related reactions (27%), pyrexia (24%), hypokalemia (15%), constipation (15%), rash (12%), abdominal pain (12%), CMV infection (12%), diarrhea (12%), lymphocytosis (12%), cough (12%), irritability (12%), tachycardia (12%), and tachypnea (12%).

HLH/MAS

Serious adverse reactions were reported in 12 patients (31%), with the most common serious adverse reaction being pneumonia (5%). Fatal adverse reactions occurred in two patients (5%) and included multiple organ dysfunction and circulatory shock.

The most common adverse reactions (≥10%) for Gamifant included viral infection (44%), rash (21%), anemia (18%), leukopenia (15%), thrombosis (15%), bacterial infections (13%), headache (13%), hyperglycemia (13%), infusion-related reactions (13%), abdominal pain (10%), hypertension (10%), pyrexia (10%), and thrombocytopenia (10%).

References

  1. Gamifant (emapalumab-lszg) prescribing information. Stockholm, Sweden: Sobi, Inc. 2024.
  2. Jordan MB, Allen CE, Weitzman S, Filipovich AH, McClain KL. How I treat hemophagocytic lymphohistiocytosis. Blood. 2011;118(15):4041-4052. doi:10.1182/blood-2011-03-278127
  3. Sepulveda FE, de Saint Basile G. Hemophagocytic syndrome: primary forms and predisposing conditions. Curr Opin Immunol. 2017;49:20-26. doi:10.1016/j.coi.2017.08.004
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  2. Dosing and Administration

TAILOR Gamifant TREATMENT TO YOUR PATIENT’S NEEDS

Gamifant is administered1:

  • By intravenous (IV) infusion over 1 hour twice a week (every 3-4 days)
  • Until the patient no longer requires therapy for the treatment of primary hemophagocytic lymphohistiocytosis (HLH), until hematopoietic stem cell transplantation (HSCT) is performed, or until unacceptable toxicity is reached
    • If conditioning is required, Gamifant can be administered throughout conditioning until HSCT is successfully performed
    • If primary HLH symptoms recur, Gamifant can be readministered
  • Concomitantly with dexamethasone at a starting daily dose of at least 5 to 10 mg/m2 the day before Gamifant treatment begins
  • Using a gamma-irradiated or ethylene oxide–sterilized, latex-free, polyvinyl chloride (PVC)-free syringe, or
  • Using a non-PVC polyolefin infusion bag

Gamifant offers flexibility of dosing1

The recommended starting dose of Gamifant is 1 mg/kg.1

Gamifant can be incrementally titrated upward or downward according to the clinician's assessment of patient response. After the patient’s clinical condition is stabilized, decrease the dose to the previous level to maintain clinical response until HSCT.1

Dexamethasone can be tapered according to the judgment of the treating physician to help reduce the risk of overexposure. A steady reduction in dexamethasone dosage was achieved in the Gamifant pivotal trial.1

From a 1 mg/kg starting dose, Gamifant can be titrated to 3 mg/kg on or after day 3, 6 mg/kg on or after day 6, and a maximum dose of 10 mg/kg on or after day 9

Drug interactions

The formation of CYP450 enzymes may be suppressed by increased levels of cytokines (such as interferon gamma [IFNγ]) during chronic inflammation. By neutralizing IFNγ, use of Gamifant may normalize CYP450 activities which may reduce the efficacy of drugs that are CYP450 substrates due to increased metabolism. Upon initiation or discontinuation of concomitant Gamifant, monitor for reduced efficacy and adjust dosage of CYP450 substrate drugs as appropriate.

Response criteria for dose increase1

After initiating Gamifant, monitor patients’ response to determine whether a dose increase may be needed based on clinical assessment of unsatisfactory improvement

Fever Persistence or recurrence
Platelet Count
  • If baseline <50,000/mm3 and no improvement to >50,000/mm3
  • If baseline >50,000/mm3 and <30% improvement
  • If baseline >100,000/mm3 and decrease to <100,000/mm3
Neutrophil Count
  • If baseline <500/mm3 and no improvement to >500/mm3
  • If baseline >500-1000/mm3 and decrease to <500/mm3
  • If baseline 1000-1500/mm3 and decrease to <1000/mm3
Ferritin
  • If baseline ≥3000 ng/mL and <20% decrease
  • If baseline <3000 ng/mL and any increase to >3000 ng/mL
Splenomegaly Any worsening
Coagulopathy Both D-dimer and fibrinogen must apply
  • D-dimer: if abnormal at baseline and no improvement
  • Fibrinogen (mg/dL): if baseline levels ≤100 mg/dL and no improvement or if baseline levels >100 mg/dL and any decrease to <100 mg/dL
dosing & Administration guide

Contact A HEALTH SYSTEMS DIRECTOR FOR ADDITIONAL QUESTIONS

Target-mediated clearance

Gamifant exhibits target-mediated clearance dependent on IFNγ production, which can vary between and within patients as a function of time and can affect the recommended dosage.1

  • The pharmacokinetics of emapalumab-lzsg were evaluated in healthy adult subjects and in patients with primary HLH1
  • Gamifant steady-state is achieved by the seventh infusion when the IFNγ production is moderate. At high IFNγ production, steady-state is reached earlier due to a shorter half-life1
  • The half-life of Gamifant is approximately 22 days in healthy subjects, and ranged from 2.5 to 18.9 days in patients with primary HLH1

Dosing flexibility is especially important given this variability, and dose adjustments may be needed to neutralize IFNγ concentrations in each patient.

The recommended starting dose of Gamifant is 1 mg/kg.1

  • If the same clinical and laboratory criteria still apply after incremental titration, the dose of Gamifant may continue to increase to a maximum of 10 mg/kg1
  • Titration should be based on clinical and lab parameters interpreted as unsatisfactory1
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Discover the storm

Primary hemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory condition that mostly affects children, but can also occur in adults and teenagers.2,3

Gamifant controls the inflammatory symptoms of primary HLH by neutralizing IFNγ.1

Dosing and Administration

Gamifant is given as an intravenous infusion twice per week until the patient no longer requires therapy for the treatment of HLH or until hematopoietic stem cell transplantation (HSCT) is performed.1

ORDERING GAMIFANT

Contact your Sobi Health Systems Director to understand Gamifant ordering options. Connect with your Reimbursement Manager for your individual patient's insurance coverage policy and reimbursement pathway.

Help through the storm

Here you’ll find tools and resources to help your patients and their families.