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Velaglucerase Alfa

Medically reviewed on Nov 15, 2018


(vel a GLOO ser ase AL fa)

Index Terms

  • Gene-Activated Human Acid-Beta-Glucosidase
  • GlcCerase

Dosage Forms

Excipient information presented when available (limited, particularly for generics); consult specific product labeling.

Solution Reconstituted, Intravenous [preservative free]:

Vpriv: 400 units (1 ea)

Brand Names: U.S.

  • Vpriv

Pharmacologic Category

  • Enzyme


Velaglucerase alfa, which contains the same amino acid sequence as endogenous glucocerebrosidase, catalyzes the hydrolysis of glucocerebroside to glucose and ceramide in the lysosome. In patients with type 1 Gaucher’s disease, glucocerebrosidase deficiency results in accumulation of glucocerebroside in macrophages, thereby causing the associated signs and symptoms. Velaglucerase alfa is used to diminish hepatosplenomegaly and improve anemia, thrombocytopenia, and bone disease.


Vd: Steady-state: 0.08-.0.11 L/kg

Half-Life Elimination

11-12 minutes

Use: Labeled Indications

Gaucher disease: For long-term enzyme replacement therapy for pediatric and adult patients with type 1 Gaucher disease.


There are no contraindications listed in the manufacturer’s labeling.

Dosing: Adult

Note: Pretreatment with antihistamines and/or corticosteroids can be considered for prevention of subsequent infusion reactions in patients with hypersensitivity reactions requiring symptomatic treatment; during clinical studies, patients were not routinely premedicated prior to infusion.

Gaucher’s disease (type 1): IV: 60 units/kg administered every 2 weeks; adjust dose based upon disease activity (range: 15-60 units/kg evaluated in clinical trials)

Note: When switching from imiglucerase to velaglucerase alfa in stable patients, initiate treatment 2 weeks after the last imiglucerase dose and at the same dose.

Dosing: Geriatric

Refer to adult dosing.

Dosing: Pediatric

Note: Pretreatment with antihistamines and/or corticosteroids can be considered for prevention of subsequent infusion reactions in patients with hypersensitivity reactions requiring symptomatic treatment; during clinical studies, patients were not routinely premedicated prior to infusion.

Gaucher’s disease (type 1): Children ≥4 years and Adolescents: Refer to adult dosing.

Dosing: Renal Impairment

No dosage adjustment provided in manufacturer's labeling.

Dosing: Hepatic Impairment

No dosage adjustment provided in manufacturer's labeling.


Reconstitute the vials with 4.3 mL of SWFI to a final concentration of 100 units/mL. Gently mix vials; do not shake. The solution for infusion should be further diluted in 100 mL of NS. Slight flocculation may occur; this is acceptable for administration.


IV: Infuse over 1 hour; use an inline, low protein-binding 0.2 micron filter during infusion. Do not infuse other products in the same infusion tubing.


Store intact vials at 2°C to 8°C (36°F to 46°F). Once reconstituted, the product should be used immediately. If immediate use is not possible, the reconstituted or diluted product may be stored for up to 24 hours at 2°C to 8°C (36°F to 46°F). The infusion should be completed within 24 hours of reconstitution. Do not freeze. Protect from light. Discard any unused solution.

Drug Interactions

There are no known significant interactions.

Adverse Reactions


Central nervous system: Headache (30% to 35%), dizziness (8% to 22%), fatigue (≤15%), weakness (≤15%)

Gastrointestinal: Abdominal pain (15% to 19%)

Hematologic & oncologic: Prolonged partial thromboplastin time (5% to 11%; more common in children)

Hypersensitivity: Hypersensitivity reaction (23% to 52%)

Neuromuscular & skeletal: Back pain (17% to 18%), arthralgia (8% to 15%; knee)

Miscellaneous: Fever (13% to 22%; more common in children)

1% to 10%:

Cardiovascular: Flushing (>2%), hypertension (>2%), hypotension (>2%), tachycardia (>2%)

Dermatologic: Skin rash ( >2%; more common in children), urticaria (>2%)

Gastrointestinal: Nausea (6% to 10%)

Immunologic: Immunogenicity (2%)

Neuromuscular & skeletal: Ostealgia (>2%)

<1%, postmarketing, and/or case reports: Anaphylaxis, chest discomfort, dyspnea, pruritus


Concerns related to adverse effects:

• Antibody formation: The development of IgG antibodies has been reported; the clinical significance is unknown. Patients with an immune response to other enzyme replacement therapies who are switching to velaglucerase alfa should be monitored for antibody development.

• Hypersensitivity reactions: Use with caution in patients who have exhibited hypersensitivity reactions to velaglucerase alfa or other enzyme replacement therapies. Anaphylaxis has occurred; appropriate medical support should be readily available in the event of a serious reaction. The most common hypersensitivity reactions reported in clinical trials include asthenia, dizziness, fatigue, fever, headache, hyper-/hypotension, nausea, and pyrexia. Most reactions were mild and occurred during the first 6 months of treatment. Management strategies of more severe reactions include symptomatic treatment, pretreatment with antihistamines, antipyretics, and/or corticosteroids, and slowing of the infusion rate. Treatment should be discontinued if anaphylaxis or other acute reactions occur.

Monitoring Parameters

Disease monitoring: CBC, liver enzymes, IgG antibodies; MRI, CT, or US of liver and spleen; bone density studies; monitor antibodies in those patients who developed antibodies to other enzyme replacement therapies

Pregnancy Risk Factor


Pregnancy Considerations

Teratogenic 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, may occur.

Patient Education

• Discuss specific use of drug and side effects with patient as it relates to treatment. (HCAHPS: During this hospital stay, were you given any medicine that you had not taken before? Before giving you any new medicine, how often did hospital staff tell you what the medicine was for? How often did hospital staff describe possible side effects in a way you could understand?)

• Patient may experience abdominal pain, back pain, or joint pain. Have patient report immediately to prescriber signs of infusion reaction, angina, severe nausea, vomiting, loss of strength and energy, tachycardia, shortness of breath, vision changes, dizziness, passing out, or headache (HCAHPS).

• Educate patient about signs of a significant reaction (eg, wheezing; chest tightness; fever; itching; bad cough; blue skin color; seizures; or swelling of face, lips, tongue, or throat). Note: This is not a comprehensive list of all side effects. Patient should consult prescriber for additional questions.

Intended Use and Disclaimer: Should not be printed and given to patients. This information is intended to serve as a concise initial reference for health care professionals to use when discussing medications with a patient. You must ultimately rely on your own discretion, experience, and judgment in diagnosing, treating, and advising patients.

Further information

Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.