Version July 2025
Patients treated with enzyme replacement therapies have experienced life-threatening hypersensitivity reactions, including anaphylaxis. Anaphylaxis has occurred during the early course of enzyme replacement therapy and after extended duration of therapy. Initiate taliglucerase alfa in a healthcare setting with appropriate medical monitoring and support measures, including access to cardiopulmonary resuscitation equipment. If a severe hypersensitivity reaction (eg, anaphylaxis) occurs, discontinue taliglucerase alfa and immediately initiate appropriate medical treatment, including use of epinephrine. Inform patients of the symptoms of life-threatening hypersensitivity reactions, including anaphylaxis, and to seek immediate medical care should symptoms occur.
Dosage guidance:
Safety: Pretreatment with antihistamines and/or corticosteroids can be considered for prevention of infusion reactions in patients with previous hypersensitivity reactions to taliglucerase alfa or other enzyme replacement therapies. Initiate in a health care setting with appropriate medical monitoring and support measures, including CPR equipment, readily available during administration.
Gaucher disease, type 1:
Note: Efficacy of taliglucerase is limited to short- and long-term improvements in skeletal deterioration, visceromegaly, and hematologic abnormalities of Gaucher disease type 1; positive effects on neurologic manifestations have not been observed (Ref).
Treatment naive:
Children ≥2 years and Adolescents: Limited data available in ages 2 to <4 years: IV: 60 units/kg/dose every 2 weeks; use actual body weight to calculate dose; adjust based on disease activity; mean final dose following 6 years of therapy: 47 units/kg/dose (in both treatment-naive patients and those switched from imiglucerase therapy) (Ref).
Conversion from imiglucerase to taliglucerase:
Children ≥2 years and Adolescents: Limited data available in ages 2 to <4 years: IV: Initiate taliglucerase alfa at the same previously stable imiglucerase dose, starting 2 weeks after last imiglucerase dose (Ref).
Gaucher disease, type 3:
Note: Efficacy of taliglucerase is limited to short- and long-term improvements in skeletal deterioration, visceromegaly, and hematologic abnormalities of Gaucher disease type 3; positive effects on neurologic manifestations have not been observed (Ref).
Children ≥2 years and Adolescents: Limited data available: IV: 30 to 60 units/kg/dose every 2 weeks; reported dosing range: 9 to 67 units/kg/dose; use actual body weight to calculate dose; adjust based on disease activity (Ref). Note: Based on experience in patients with type I Gaucher disease, when switching from imiglucerase to taliglucerase alfa, consider initiating taliglucerase at the same previously stable imiglucerase dose, starting 2 weeks after last imiglucerase dose (Ref).
There are no dosage adjustments provided in the manufacturer’s labeling.
There are no dosage adjustments provided in the manufacturer’s labeling.
(For additional information see "Taliglucerase alfa (glucocerebrosidase): Drug information")
Dosage guidance:
Clinical considerations: Pretreatment with antihistamines and/or corticosteroids can be considered for prevention of subsequent infusion reactions in patients with previous hypersensitivity reactions to taliglucerase alfa or other enzyme replacement therapies; during clinical studies, patients were not routinely premedicated prior to infusion.
Gaucher disease (type 1): IV: Dosing based on actual body weight: 60 units/kg every 2 weeks; dosing is individualized based on disease severity.
Conversion from imiglucerase: Initiate taliglucerase alfa using the patient's same previous imiglucerase dose and administer every 2 weeks. Note: Conversion to taliglucerase alfa is based on a single study of patients stabilized on a biweekly imiglucerase dose for ≥6 months
There are no dosage adjustments provided in the manufacturer's labeling.
There are no dosage adjustments provided in the manufacturer's labeling.
The following adverse drug reactions and incidences are derived from product labeling unless otherwise specified.
>10%:
Hypersensitivity: Hypersensitivity reaction
Immunologic: Antibody development (including neutralizing)
Nervous system: Headache
Neuromuscular & skeletal: Arthralgia
1% to 10%:
Cardiovascular: Flushing
Dermatologic: Pruritus, urticaria
Gastrointestinal: Abdominal pain, nausea, vomiting
Hypersensitivity: Anaphylaxis
Nervous system: Dizziness, fatigue
Neuromuscular & skeletal: Limb pain
Frequency not defined: Hypersensitivity: Angioedema
Postmarketing:
Dermatologic: Fixed drug eruption (type III immune mediated)
Gastrointestinal: Diarrhea
Neuromuscular & skeletal: Back pain
There are no contraindications listed in the US labeling.
Canadian labeling: Severe hypersensitivity to taliglucerase alfa or any component of the formulation.
Concerns related to adverse effects:
• Antibody formation: The development of IgG anti-drug antibodies (ADA) has been reported; the clinical significance is unknown; patients who develop immune or infusion reactions to taliglucerase alfa or who have had an immune response to other enzyme replacement therapies and who are switching to taliglucerase alfa should be monitored for ADA; the presence of antibodies may be related to a higher risk of hypersensitivity reactions.
• CNS effects: Dizziness and fatigue have been observed with therapy; caution patients about performing dangerous tasks (eg, driving, operating machinery).
• Hypersensitivity reactions: Anaphylaxis has occurred early in therapy and after extended use; appropriate medical support should be readily available in the event of a serious reaction. The most common hypersensitivity reactions reported in clinical trials include angioedema, cough, chest tightness, erythema, flushing, pruritus, nausea, rash, throat irritation, and vomiting. Discontinue infusion and institute appropriate therapy if hypersensitivity or other acute reaction occurs. Use with caution in patients who have exhibited hypersensitivity reactions to taliglucerase alfa or other enzyme replacement therapies. Incidence is increased in patients with antibodies to taliglucerase alfa.
Other warnings/precautions:
• Experienced personnel: Administration should be supervised by a health care provider knowledgeable in the management of hypersensitivity reactions, including anaphylaxis.
In a clinical trial evaluating the safety of taliglucerase alfa, treatment-naïve pediatric patients experienced a higher incidence of vomiting compared to treatment-naïve adults (44% vs 6%). Vomiting may be a symptom of hypersensitivity; monitor patients carefully.
Excipient information presented when available (limited, particularly for generics); consult specific product labeling.
Solution Reconstituted, Intravenous [preservative free]:
Elelyso: 200 units (1 ea) [contains polysorbate 80]
No
Solution (reconstituted) (Elelyso Intravenous)
200 unit (per each): $1,078.62
Disclaimer: A representative AWP (Average Wholesale Price) price or price range is provided as reference price only. A range is provided when more than one manufacturer's AWP price is available and uses the low and high price reported by the manufacturers to determine the range. The pricing data should be used for benchmarking purposes only, and as such should not be used alone to set or adjudicate any prices for reimbursement or purchasing functions or considered to be an exact price for a single product and/or manufacturer. Medi-Span expressly disclaims all warranties of any kind or nature, whether express or implied, and assumes no liability with respect to accuracy of price or price range data published in its solutions. In no event shall Medi-Span be liable for special, indirect, incidental, or consequential damages arising from use of price or price range data. Pricing data is updated monthly.
Excipient information presented when available (limited, particularly for generics); consult specific product labeling.
Solution Reconstituted, Intravenous:
Elelyso: 200 units (1 ea) [contains polysorbate 80]
Product access is restricted to the Gaucher Personal Support (GPS) program. Healthcare providers and patients may obtain additional information by contacting the GPS program at 855-353-5976.
Note: Monitor patients closely for infusion-related or hypersensitivity reactions. Initiate infusion in a health care setting that includes cardiopulmonary monitoring and can provide resuscitation if necessary.
Parenteral: IV: Infusion rate depends on weight (minimum infusion time: 60 minutes). Infuse through an in-line low protein-binding 0.2 micron filter. Observe patient for 3 hours after the start of each infusion.
Weight <30 kg: Infuse at a rate of 1 mL/minute.
Weight ≥30 kg: Initiate infusion at a rate of 1 mL/minute; the infusion rate may be increased if tolerated to a maximum of 2 mL/minute.
Rate adjustment for hypersensitivity:
Infusion-related reactions or mild/moderate hypersensitivity reactions: Treatment should be based on the severity of the reaction and may include temporarily interrupting the infusion, decreasing the infusion rate, and/or administration of antihistamines, antipyretics, and/or corticosteroids (Ref). Consider pretreatment with antihistamines and/or corticosteroids for subsequent infusions; closely monitor when readministering.
Anaphylaxis or severe hypersensitivity: Discontinue therapy immediately for severe hypersensitivity reactions or anaphylaxis and treat appropriately. If the decision is made to readminister after anaphylaxis, consider premedication with antihistamines and/or corticosteroids for subsequent infusions and reduction of infusion rate; appropriately trained personnel and emergency medications should be readily available. Monitor patient closely for recurrence.
IV: Administer IV over a minimum infusion time of 60 minutes (usual infusion time: 60 to 120 minutes). Administer using a low protein-binding infusion set with a 0.2-micron in-line filter.
Initiate infusion at a rate of 1.2 mL/minute; rate may be increased, but not exceed 2.2 mL/minute based on patient tolerance.
For mild hypersensitivity reactions, decrease rate or temporarily hold taliglucerase alfa, and/or administer antihistamines, antipyretics, and/or corticosteroids. For severe hypersensitivity reactions or anaphylaxis, discontinue immediately and initiate appropriate medical treatment.
Store intact vials at 2°C to 8°C (36°F to 46°F); protect from light. Do not freeze. If not used immediately, the reconstituted solution may be stored at 2°C to 8°C (36°F to 46°F) for ≤24 hours (protect from light) or at 20°C to 25°C (68°F to 77°F) for ≤4 hours (without protection from light). The solution diluted in NS for infusion may be stored at 2°C to 8°C (36°F to 46°F) for ≤24 hours; protect from light. In total, the reconstituted and diluted products can be stored for ≤24 hours. Do not freeze.
Long-term enzyme replacement therapy for patients with type 1 Gaucher disease (FDA approved in ages ≥4 years and adults); has also been used for patients with type 3 Gaucher disease.
Canadian labeling: Long-term enzyme replacement therapy for patients with type 1 Gaucher disease in pediatric patients 2 to 17 years of age and adults; long-term enzyme replacement therapy for pediatric patients 2 to 17 years of age with type 3 Gaucher disease.
None known.
Note: Interacting drugs may not be individually listed below if they are part of a group interaction (eg, individual drugs within “CYP3A4 Inducers [Strong]” are NOT listed). For a complete list of drug interactions by individual drug name and detailed management recommendations, use the drug interactions program
There are no known significant interactions.
Adverse effects were not observed in animal reproduction studies. Pregnancy may exacerbate existing type I Gaucher disease or result in new symptoms. Women with type I Gaucher disease have an increased risk of spontaneous abortion if disease is not well controlled. Adverse pregnancy outcomes, including hepatosplenomegaly and thrombocytopenia (which may result in increased bleeding and postpartum hemorrhage requiring transfusion), may occur.
Signs/symptoms of anaphylaxis and hypersensitivity (for 3 hours after start of infusion); hemoglobin, platelet count, ECG and echocardiogram (baseline, every 12 months until clinical goals achieved, then every 12 to 24 months) (Baldellou 2004; Charrow 2004), IgG anti-drug antibody formation (in patients who experience, or previously experienced, immune or infusion reactions to enzyme replacement therapy, bone density studies; liver function tests; abdominal ultrasound (hepatomegaly, hepatosplenomegaly); disease biomarkers.
Taliglucerase alfa is an analogue of glucocerebrosidase; it is produced by recombinant DNA technology using plant (carrot) cell culture. Glucocerebrosidase is an enzyme deficient in Gaucher's disease. It is needed to catalyze the hydrolysis of glucocerebroside to glucose and ceramide, thereby reducing liver and spleen size and improving anemia and thrombocytopenia.
Distribution: Vss: Pediatric patients: 8.8 to 14.9 L; Adults: 10.7 to 11.7 L
Half-life elimination: Pediatric patients: 33 to 37 minutes; Adults: 19 to 29 minutes (dose-dependent; increased with higher doses)
