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Velaglucerase alfa parenteral

Updated 2 Feb 2023 | Gaucher's disease


Parenteral formulations of velaglucerase alfa.

Drugs List

  • velaglucerase alfa 400unit powder for solution for infusion
  • VPRIV 400unit powder for solution for infusion
  • Therapeutic Indications


    Enzyme replacement therapy in patients with Type 1 Gaucher disease


    Dosage should be individualised for each patient.

    Patients switching from imiglucerase enzyme replacement therapy for type 1 Gaucher disease to velaglucerase alfa, should use the same dose and frequency.


    The recommended dose is 60units/kg every other week.

    Clinical studies have evaluated doses ranging from 15 to 60units/kg every other week.


    Children aged 4 to 18 years
    The recommended dose is 60units/kg every other week.

    Clinical studies have evaluated doses ranging from 15 to 60units/kg every other week.


    For intravenous infusion.


    Children under 4 years
    Hereditary fructose intolerance

    Precautions and Warnings

    Restricted sodium intake
    Glucose-galactose malabsorption syndrome

    Sodium content of formulation may be significant
    Consider premedication with antihistamine and/or corticosteroid
    Treatment to be initiated and supervised by a specialist
    Preparation contains sucrose
    Do not use if solution is discoloured or particulates are apparent
    Record name and batch number of administered product
    Resuscitation facilities must be immediately available
    Monitor for hypersensitivity reactions during infusion
    Monitor for the presence of antibodies
    Advise patient to stop therapy & contact Dr if hypersensitivity signs occur
    Discontinue if serious allergic or anaphylactic reaction occurs

    Home administration under the supervision of a healthcare professional may be considered only for patients who have received at least three infusions and were tolerating them well.

    In clinical studies only 1% of the patients develop IgG antibodies, and no patients developed IgE antibodies to velaglucerase alfa. In case of severe infusion related reactions and lack or loss of effect, the patient should be evaluated for the presence of antibodies and the results reported.

    The majority of hypersensitivity reactions usually occur up to 12 hours post infusion. The most frequently reported hypersensitivity reactions include nausea, rash, dyspnoea, back pain, chest discomfort, urticaria, arthralgia and headache. Vomiting as a side effect can be serious and severe and often occurs during the infusion and for 24 hours after the infusion.

    Pregnancy and Lactation


    Use velaglucerase alfa with caution during pregnancy.

    The manufacturer recommends that pregnant women may experience a period of increased disease activity, therefore a risk-benefit assessment for each pregnancy is required. Close monitoring of the pregnancy and clinical manifestations of Gaucher disease is needed for the individualisation of therapy.

    At the time of writing there is limited published data regarding the use of velaglucerase alfa during pregnancy. The potential risk is unknown.


    Use velaglucerase alfa with caution during breastfeeding.

    The manufacturer advises that at the time of publication there had been no studies in breastfeeding women. It is unknown whether velaglucerase alfa is excreted into breast milk. The effects on exposed infants are unknown.

    Side Effects

    Abdominal pain
    Allergic dermatitis
    Anaphylactic reaction
    Anaphylactoid reaction
    Antibody formation
    Back pain
    Blurred vision
    Bone pain
    Chest discomfort
    Hypersensitivity reactions
    Increased partial thromboplastin time
    Infusion-related symptoms
    Rise in body temperature
    Upper abdominal pain


    It is strongly recommended that the UK National Poisons Information Service be consulted on cases of suspected or actual overdose where there is doubt over the degree of risk or about appropriate management.

    The following number will direct the caller to the relevant local centre (0844) 892 0111

    Information may be obtained if you have access to ToxBase the primary clinical toxicology database of the National Poisons Information Service. This is available via password on the internet ( ) or if this is unavailable at the backup site ( ).

    Further Information

    Last Full Review Date: November 2019

    Reference Sources

    Summary of Product Characteristics: VPRIV 400 Units powder for solution for infusion. Shire Pharmaceuticals Ireland Limited. Revised July 2020.

    NICE Evidence Services Available at: Last accessed: 09 November 2019.

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