Primary hemophagocytic lymphohistiocytosis (HLH) in patients with refractory, recurrent or progressive disease or intolerance with conventional therapy.
IV (Adults and Children): 1 mg/kg twice weekly (every 3–4 days) on Days 1 and 2; on Day 3, may ↑ dose to 3 mg/kg twice weekly (every 3–4 days) if there is unsatisfactory improvement in condition (as determined by specific criteria); on Day 6, may ↑ dose to 6 mg/kg twice weekly (every 3–4 days) if there continues to be unsatisfactory improvement in condition (as determined by specific criteria); on Day 9, may ↑ dose to 10 mg/kg twice weekly (every 3–4 days) if healthcare provider determines that, based on initial signs of response, that a further ↑ in dose may be beneficial. Continue therapy until hematopoietic stem cell transplantation or unacceptable toxicity.
Assess for tuberculosis using purified protein derivative (PPD) or IFN. release assay and evaluate patients for tuberculosis risk factors prior to starting therapy. If patient test positive or is at risk tuberculosis, administer prophylaxis. Monitor for tuberculosis, adenovirus, Epstein-Barr virus (EBV) and cytomegalovirus (CMV) every 2 wks during therapy and as clinically indicated.
Monitor for signs and symptoms of infusion-related reactions (fever, rash, erythema, hyperhidrosis) during infusion. If reactions occur, interrupt therapy and treat reaction, then infuse at slower rate.
Administer prophylaxis for herpes zoster, Pneumocystis jirovecii, and fungal infections prior to administration.
Begin dexamethasone daily of at least 5 to 10 mg/m2 the day before starting emapalumab therapy in patients not receiving dexamethasone. For patients receiving baseline dexamethasone, continue regular dose provided dose is at least 5 mg/m2 .
Intermittent Infusion: Calculate dose (mg/kg), total volume (mL) of emapalumab required and number of vials needed based on patient actual body weight. Solution is clear to slightly opalescent, colorless to slightly yellow; do not administer solutions that are cloudy, discolored or contain particulate matter. Dilution: 0.9% NaCl. Concentration: Up to 2.5 mg/mL. Do not dilute to <0.25 mg/mL. Discard unused solution. Place in syringe or infusion bag, based on volume needed. Use gamma irradiated latex-free, polyvinyl chloride (PVC)-free syringe. Do not use with ethylene oxide-sterilized syringes. Use a non-PVC polyolefin infusion bag. Diluted solution is stable for up to 4 hr if refrigerated. Allow to come to room temperature before infusion. Do not shake or freeze.
Rate: Infuse over 1 hr through an IV line with a sterile, non-pyrogenic, low-protein binding 0.2 micron in-line filter.
Y-Site Incompatibility: Do not infuse with other with other agents.
Explain purpose and schedule of emapalumab therapy to patient.
Advise patient to notify health care professional if signs and symptoms of infusion-reactions (skin redness, itching, fever, rash, excessive sweating, chills, chest pain, shortness of breath, nausea, vomiting, lightheadedness or dizziness) or infection occur.
Advise patient to avoid live or live attenuated vaccines during and up to 4 wks after last dose of therapy.
Instruct patient to notify health care professional of all Rx or OTC medications, vitamins, or herbal products being taken and consult health care professional before taking any new medications.
Advise patient to notify health care professional if pregnancy is planned or suspected or if breastfeeding.