• Primary hemophagocytic lymphohistiocytosis (HLH) in patients with refractory, recurrent or progressive disease or intolerance with conventional therapy.

Contraindicated in:

• Active infection caused by mycobacteria, Herpes zoster, or Histoplasma capsulatum.

Use Cautiously in:

• History of exposure to tuberculosis
• OB: Safety not established
• Lactation: Use while breastfeeding only if potential maternal benefit justifies potential risk to infant.

CV: hypertension, tachycardia, bradycardia, peripheral edema.

Derm: rash.

EENT: epistaxis.

F and E: hypokalemia.

GI: abdominal pain, constipation, diarrhea, GI HEMORRHAGE, vomiting.

GU: acute kidney injury.

Hemat: lymphocytosis.

Neuro: irritability.

Resp: cough, tachypnea, dyspnea.

Misc: fever, infection, infusion reactions.

Drug-Drug:

• May ↓ antibody response to live-virus or live-attenuated vaccines and ↑ risk of adverse reactions; do not administer concurrently.

• Solution for injection: 5 mg/mL;

• 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.

Gamifant

• Monoclonal antibody that binds to and neutralizes interferon gamma, which is produced in excess in HLH.

• Improvement or normalization (complete or partial) of HLH abnormalities.

Therapeutic Classification: n/a

Pharmacologic Classification: monoclonal antibodies

Absorption: IV administration results in complete bioavailability.

Distribution: Not widely distributed to tissues.

Metabolism/Excretion: Degraded into small peptides and amino acids via catabolic pathways.

Half-life: 2.5–18.9 days.

(plasma concentrations)

• 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.

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