emapalumab

REMS

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

Contraind./Precautions ⬆ ⬇

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.

Adv. Reactions/Side Effects ⬆ ⬇

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

Interactions ⬆ ⬇

Drug-drug:

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

Availability ⬆ ⬇

Solution for injection: 5 mg/mL

Route/Dosage ⬆ ⬇

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.

US Brand Names ⬆ ⬇

Action ⬆ ⬇

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

Therapeutic effects:

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

Classifications ⬆ ⬇

Therapeutic Classification: none assigned

Pharmacologic Classification: monoclonal antibodies

Pharmacokinetics ⬆ ⬇

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.

Time/Action Profile ⬆ ⬇

(plasma concentrations)

ROUTE	ONSET	PEAK	DURATION
IV	unknown	unknown	unknown

Patient/Family Teaching ⬆ ⬇

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.

section name header

Indications ⬇