Treatment of adult and pediatric (newborn and older) patients with primary hemophagocytic lymphohistiocytosis (HLH) with refractory, recurrent, or progressive disease who are intolerant of conventional HLH therapy. Off label for Macrophage Activation Syndrome.
Dose per vial
- Available as 10 and 50 mg preservative free, single use vials at a concentration of 5 mg/mL sterile solution
- Clear to slightly opalescent, colorless or slightly yellow preservative-free solution. Do not administer if discolored or foreign particulate matter is present.
- Store vials in original carton under refrigeration (2 - 8 degrees C) DO NOT FREEZE
- Protect from light
- Dilute with 0.9% NaCL injection to a concentration between 0.25 - 2.5 mg/mL. Discard any unused portion left in the vial(s).
- Gently invert the infusion bag or syringe several times to ensure complete and homogenous distribution. Do not shake.
- Reconstituted solution must be in a gamma irradiated latex-free, polvinyl chloride (PVC)-free syringe OR non-PVC polyolefin infusion bag.
- Store diluted solution of emapalumab under refrigeration between 2 - 8 degrees C for up to 16 hours. If refrigerated, allow the diluted solution to come to room temperature prior to administration.
- Solutions compatible: 0.9% NaCL
- Additives/Above Cassette compatible: No information
- Y-Site compatability: No information
- Incompatible: DO NOT infuse any other medication or solution with emapalumab.
(For approved routes of administration by nursing personnel, refer to Policy for the Administration of Intravenous Medications.)
|IV intermittent infusion||
YES. Dilute between 0.25 mg/mL and 2.5 mg/mL.
Infusion time: 1 - 2 hours
Administer through a sterile, non-pyrogenic, low- protein binding 0.2 micron inline filter.
|IV continuous infusion||NO|
(For neonatal dosages, refer to Neonatal IV Drug Manual.)
Macrophage Activation Syndrome (off label)
|Treatment Day||Emapalumab Dose|
|Subsequent treatment days||3 mg/kg twice weekly|
Emapalulab dose can be increased (or frequency between infusion shortened) guided by the clinical and laboratory response or pharmacokinetic data in the individual patient, only after receiving Novimmune's (the maker of emapalulmab) approval.
- Infusion related reactions including drug eruption, anaphylaxis, pyrexia, rash, erythema, and hyperhidrosis. Interrupt infusion for infusion reactions and institute CHEO appropriate medical management prior to continuing infusion at a slower rate.
- The most common reported adverse reactions (> 10%) include 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.
- Rare adverse reactions (< 10%) include vomiting, acute kidney injury, asthenia, bradycardia, dyspnea, gastrointestinal hemorrhage, epistaxis, and peripheral edema.
- No anaphylactic/anaphylactoid reactions or delayed hypersensitivity reactions have been observed; however, given the currently limited clinical experience, anaphylactic precautions should be taken when administering emapalumab.
- Live or attenuated vaccines should not be administered for at least 4 weeks after the last dose of emapalumab. DO NOT administer live vaccines concurrently with emapalumab.