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Generic Name: Velaglucerase Alfa
Class: Enzymes
Chemical Name: Ceramidase, glucosyl-(human HT-1080 cell)
Molecular Formula: C2532H3854N672O711S16
CAS Number: 0884604-91-5


Biosynthetic (gene activation technology origin) form of human β-glucocerebrosidase (glucosylceramidase).1 3 4 33

Uses for VPRIV

Gaucher's Disease

Long-term enzyme replacement therapy in patients with nonneuronopathic (type 1) Gaucher's disease1 5 32 33 (designated an orphan drug by FDA for this use).2

VPRIV Dosage and Administration


  • Administer under the supervision of a clinician.1


IV Administration

For solution compatibility information, see Compatibility under Stability.

Administer by IV infusion.1

Administer using an inline, low-protein-binding, 0.2-mcm particulate filter.1

Velaglucerase alfa does not contain preservatives; prepare solutions immediately before use.1 If immediate use is not possible, complete infusion within 24 hours of reconstitution.1


Determine number of vials to be reconstituted based on patient weight.1

Reconstitute appropriate number of vials containing 200 or 400 units of velaglucerase alfa lyophilized powder with 2.2 or 4.3 mL of sterile water for injection, respectively, to provide a solution containing 100 units/mL.1 Mix gently; do not shake vial.1


Withdraw appropriate dose from reconstituted vials and dilute with 100 mL of 0.9% sodium chloride injection.1 Mix solution gently; do not shake.1

Rate of Administration

Administer over 1 hour.1


Pediatric Patients

Gaucher's Disease

Children and adolescents 4–17 years of age: Initially, 60 units/kg every 2 weeks.1

Adjust dosage based on achievement and maintenance of patient's therapeutic goals; dosages ranging from 15–60 units/kg every 2 weeks were evaluated in clinical trials.1

Patients receiving imiglucerase can be switched to velaglucerase alfa at an equivalent dosage.1


Gaucher's Disease

Initially, 60 units/kg every 2 weeks.1

Adjust dosage based on achievement and maintenance of the patient's therapeutic goals; dosages ranging from 15–60 units/kg every 2 weeks were evaluated in clinical trials.1

Patients receiving imiglucerase can be switched to velaglucerase alfa at an equivalent dosage.1

Cautions for VPRIV


  • No known contraindications.1


Sensitivity Reactions

Hypersensitivity Reactions

Hypersensitivity reactions reported.1

Carefully reevaluate therapy if substantial clinical evidence of hypersensitivity develops.1 Treat severe hypersensitivity reactions in accordance with current emergency practice standards; appropriate medical support should be readily available.1

Use with caution in patients who have exhibited manifestations of hypersensitivity reactions to velaglucerase alfa, any ingredient in the formulation, or other enzyme replacement therapy.1

Infusion-related Reactions

Infusion-related reactions (e.g., headache, dizziness, hypotension, hypertension, nausea, fatigue/asthenia, pyrexia) reported.1

Depending on severity, manage infusion-related reactions by slowing the infusion rate, providing appropriate medical treatment (e.g., antihistamines, antipyretics, and/or corticosteroids), and/or interrupting and then resuming treatment at a slower infusion rate.1

Pretreatment with antihistamines and/or corticosteroids may prevent subsequent reactions in patients who have experienced infusion-related reactions requiring treatment.1

Antibody Formation

Development of IgG antibodies to velaglucerase alfa reported.1 Effect on risk of infusion reactions is not known.1

Continue to monitor for antibodies in patients with an immune response to other enzyme replacement therapies who are switched to velaglucerase alfa.1

Specific Populations


Category B.1


Not known whether velaglucerase alfa is distributed into milk.1 Use with caution.1

Pediatric Use

Safety and efficacy not established in children <4 years of age.1

Geriatric Use

Insufficient experience in patients ≥65 years of age to determine whether geriatric patients respond differently than younger adults; select dosage with caution.1

Common Adverse Effects

Infusion-related reactions, headache, upper respiratory tract infection, dizziness, pyrexia, abdominal pain, back pain, joint (knee) pain, asthenia/fatigue, prolonged aPTT, nausea.1

Upper respiratory tract infection, rash, prolonged aPTT, and pyrexia reported more frequently in pediatric patients than in adults.1

Interactions for VPRIV

Formal drug interaction studies not performed to date.1

VPRIV Pharmacokinetics



Accumulation not observed in patients receiving 60 units/kg every 2 weeks for 37 weeks.1



Not known whether velaglucerase alfa distributes into milk.1



11–12 minutes.1




Powder for Injection

2–8°C; protect from light.1 Do not freeze.1

Following reconstitution or dilution, 2–8°C for up to 24 hours; protect from light; do not freeze.1


For information on systemic interactions resulting from concomitant use, see Interactions.


Solution Compatibility1


Sodium chloride 0.9%


  • Replaces the deficient endogenous enzyme (β-glucocerebrosidase; glucosylceramidase) in patients with Gaucher’s disease.1 3 4

  • Enzyme replacement therapy for type 1 Gaucher's disease increases the degradation of glucosylceramide (glucocerebroside) in macrophages1 3 4 5 7 8 10 26 27 by hydrolyzing the β-glycoside linkage of glucocerebroside to glucose and ceramide (N-acylsphingosine) with resultant reduction in liver and spleen size, amelioration of anemia and thrombocytopenia, decreased bone pain, decreased cachexia, and increased bone remineralization over a period of several years.4 6 7 8 9 10 11 12 13 14 15 16 17 18 19

Advice to Patients

  • Risk of hypersensitivity and infusion-related reactions.1

  • Importance of informing clinicians of existing or contemplated concomitant therapy, including prescription and OTC drugs and herbal supplements, as well as any concomitant illnesses.1

  • Importance of women informing clinicians if they are or plan to become pregnant or plan to breast-feed.1

  • Importance of informing patients of other important precautionary information. (See Cautions.)1


Excipients in commercially available drug preparations may have clinically important effects in some individuals; consult specific product labeling for details.

Please refer to the ASHP Drug Shortages Resource Center for information on shortages of one or more of these preparations.

Velaglucerase Alfa


Dosage Forms


Brand Names



For injection, for IV infusion

200 units



400 units



AHFS DI Essentials. © Copyright 2017, Selected Revisions February 15, 2013. American Society of Health-System Pharmacists, Inc., 4500 East-West Highway, Suite 900, Bethesda, Maryland 20814.


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11. Reviewers’ comments (personal observations) on alglucerase.

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