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Velaglucerase Alfa

Brand name: VPRIV
Drug class: Enzymes
Chemical name: Ceramidase, glucosyl-(human HT-1080 cell)
Molecular formula: C2532H3854N672O711S16
CAS number: 0884604-91-5

Medically reviewed by on Mar 14, 2022. Written by ASHP.


Biosynthetic (gene activation technology origin) form of human β-glucocerebrosidase (glucosylceramidase).

Uses for Velaglucerase Alfa

Gaucher Disease

Long-term enzyme replacement therapy in patients with nonneuronopathic (type 1) Gaucher disease (designated an orphan drug by FDA for this use).

Velaglucerase Alfa Dosage and Administration


  • Administer under the supervision of a clinician.


IV Administration

For solution compatibility information, see Compatibility under Stability.

Administer by IV infusion.

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

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


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

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


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

Rate of Administration

Administer over 1 hour.


Pediatric Patients

Gaucher Disease

Children and adolescents ≥4 years of age: 60 units/kg every 2 weeks.

Adjust dosage based on achievement and maintenance of patient's therapeutic goals.

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


Gaucher Disease

60 units/kg every 2 weeks.

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.

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

Cautions for Velaglucerase Alfa


  • No known contraindications.


Sensitivity Reactions

Hypersensitivity Reactions

Hypersensitivity reactions, including anaphylaxis, reported.

Manage hypersensitivity reactions based on severity. If anaphylaxis or other severe hypersensitivity occurs, discontinue velaglucerase alfa immediately and initiate appropriate therapy. For mild reactions, may reduce infusion rate, temporarily interrupt infusion, and/or administer antihistamines, antipyretics, and/or corticosteroids. Ensure that appropriate medical support is readily available during administration.

Premedication with antihistamines and/or corticosteroids may prevent subsequent reactions in patients who have experienced a reaction to velaglucerase alfa or other enzyme replacement therapy.

Antibody Formation

As with all therapeutic proteins, there is a potential for immunogenicity with velaglucerase alfa therapy. Development of IgG antibodies to velaglucerase alfa reported in at least one patient. Relationship between such antibodies and hypersensitivity risk not known.

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

Specific Populations


Category B. No adequate and well-controlled studies in pregnant women; use during pregnancy only if clearly needed. Animal studies suggest no evidence of fetal harm.


Not known whether velaglucerase alfa is distributed into milk. Use with caution. Consider the known benefits of breast-feeding along with the woman's clinical need for velaglucerase alfa and any potential adverse effects of the drug or disease on the infant.

Pediatric Use

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

Geriatric Use

Clinical response and adverse effects in geriatric patients appear to be similar to those of younger adults; in general, select dosage with caution in geriatric patients.

Common Adverse Effects

Hypersensitivity reactions, headache, dizziness, pyrexia, abdominal pain, back pain, joint pain, asthenia/fatigue, prolonged aPTT, nausea.

Interactions for Velaglucerase Alfa

Formal drug interaction studies not performed to date.

Velaglucerase Alfa Pharmacokinetics



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



Not known whether velaglucerase alfa distributes into milk.



11–12 minutes.




Powder for Injection

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

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


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


Solution Compatibility1


Sodium chloride 0.9%


  • Replaces the deficient endogenous enzyme (glucocerebrosidase) in patients with Gaucher disease.

  • Gaucher disease is an autosomal recessive lysosomal storage disorder characterized by a deficiency of the enzyme glucocerebrosidase, which results in accumulation of glucocerebroside within the lysosomes of macrophages in the liver, spleen, bone marrow, and other organs; clinical manifestations include hepatosplenomegaly, anemia, thrombocytopenia, and skeletal complications (e.g., osteopenia, osteonecrosis, progressive joint destruction, fractures).

  • Enzyme replacement therapy in patients with type 1 Gaucher disease increases the degradation of glucocerebroside to glucose and ceramide, 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.

Advice to Patients

  • Risk of hypersensitivity reactions, including anaphylaxis. Advise patients of the possible signs and symptoms of a hypersensitivity reaction and to seek immediate medical care if such manifestations occur.

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

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

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


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

400 units



AHFS DI Essentials™. © Copyright 2022, Selected Revisions March 22, 2017. American Society of Health-System Pharmacists, Inc., 4500 East-West Highway, Suite 900, Bethesda, Maryland 20814.

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