Gamifant (emapalumab-lzsg)

If there is an unsatisfactory response, the emapalumab-lzsg dose may be increased to a maximum cumulative dose of 10 mg/kg over 3 days AND the frequency may be increased to every 2 days or once daily. After improvement in the patient's clinical condition occurs, the emapalumab-lzsg dose may be reduced to the previous level; maintenance of the clinical response should be assessed. If the clinical condition is not stabilized with the maximum emapalumab-lzsg dosage, discontinuation of therapy should be considered.

The dose of emapalumab-lzsg may be increased in patients with primary HLH with unsatisfactory improvement in their clinical condition (as assessed by a healthcare provider) and at least one of the following:

*Persistent or recurrent fever

*Baseline platelet count <50,000/mm3 and no improvement to >50,000/mm3.

*Baseline platelet count >50,000/mm3 and <30% improvement.

*Baseline platelet count >100,000/mm3 and decrease to <100,000/mm3.

*Baseline neutrophil count <500/mm3 and no improvement to >500/mm3.

*Baseline neutrophil count >500-1000/mm3 and decrease to <500/mm3.

*Baseline neutrophil count 1000-1500/mm3 and decease to <1000/mm3.

*Baseline ferritin >=3000 ng/mL and <20% decrease.

*Baseline ferritin <3000 ng/mL and any increase to >3000 ng/mL.

*Worsening splenomegaly.

*Coagulopathy (both D-dimer and fibrinogen must apply):

*Abnormal D-dimer at baseline and no improvement.

*Baseline fibrinogen levels <=100 mg/dL and no improvement.

*Baseline fibrinogen levels >100 mg/dL and any decrease to <100 mg/dL.

If the dose titration criteria is met, the dose may be increased to 3 mg/kg on day 4 onwards. If patients receiving emapalumab-lzsg 3 mg/kg are determined to have unsatisfactory improvement in their clinical condition based on the criteria listed above, the dose may be increased to 6 mg/kg from day 7 onwards. If patients receiving emapalumab-lzsg 6 mg/kg are assessed by a healthcare provider who determines that a further dose increase can be of benefit based on initial signs of response, the dose may be increased to 10 mg/kg from day 10 onwards. Once the patient's clinical condition is stabilized, decrease the dose to the previous dose level to maintain clinical response.

Emapalumab-lzsg is administered by IV infusion.

Emapalumab-lzsg is commercially available as an injection concentrate containing 5 mg/mL or 25 mg/mL in single-dose vials that mustbe diluted prior to IV infusion. The 5 mg/mL and 25 mg/mL vials should not be mixed together. Emapalumab-lzsg should not be infused with other medications and other products should not be added to the infusion bag or syringe containing emapalumab-lzsg.

Calculate the dose, total volume required, and number of vials of emapalumab-lzsg needed based on the patient's actual body weight.

Emapalumab-lzsg injection is preservative-free. Discard any unused portion left in the single-use vials after preparation in accordance with local requirements.

Store unopened vials of emapalumab-lzsg injection concentrate between 2-8degreesC in the original carton to protect from light; do not freeze or shake.

No dosing information is available for this tab.

No dosing information is available for this tab.

No drug-drug interaction studies have been conducted with emapalumab-lzsg.

There are no contraindication interactions for this drug.

There are no severe interactions for this drug.

There are no moderate interactions for this drug.

*None.

There are no contraindication contraindications for this drug.

There are no severe contraindications for this drug.

There are no moderate contraindications for this drug.

The most common adverse reactions reported in >=20% of patients with primary HLH receiving emapalumab-lzsg in clinical trials included infections, hypertension, infusion-related reactions, and pyrexia.

The most common adverse reactions reported in >=20% of patients with HLH/MAS in Still's disease receiving emapalumab-lzsg in clinical trials were viral infections, including CMV infection or reactivation, and rash.

There are no severe adverse reactions for this drug.

There are no less severe adverse reactions for this drug.

No enhanced messaging available.

Safety and effectiveness of emapalumab-lzsg have been established in pediatric patients (newborn and older) with primary HLH that is reactivated or refractory to conventional therapies and with HLH/MAS in Still's disease (including juvenile idiopathic arthritis) with an inadequate response to glucocorticoids. Use of emapalumab-lzsg is supported by results from 3 single-arm trials, which included a total of 57 pediatric patients: 27 with primary HLH and 30 with HLH/MAS in Still's disease. These studies included pediatric patients ranging in age from newborn to <17 years of age.

No data are available to inform a drug-associated risk of adverse developmental outcomes with emapalumab-lzsg use in pregnancy. In an animal reproduction study, a murine surrogate anti-mouse IFNgamma antibody administered to pregnant mice throughout gestation crossed the placental barrier, and no fetal harm was observed.

It is not known whether emapalumab-lzsg is distributed into human milk. The effects of the drug on breast-fed infants or on milk production are also unknown. However, published data suggest that only limited amounts of therapeutic antibodies are found in breast milk and they do not enter the neonatal and infant circulations in substantial amounts. The manufacturer states that the developmental and health benefits of breast-feeding should be considered along with the mother's clinical need for emapalumab-lzsg and any potential adverse effects on the breast-fed infant from the drug or from the underlying maternal condition.

The manufacturer makes no specific dosage recommendations for geriatric patients.

Clinical trials of emapalumab-lzsg did not include sufficient numbers of patients >=65 years of age to determine whether they respond differently than younger patients. Other reported clinical experience has not identified differences in responses between the elderly and younger patients.