Vpriv (velaglucerase alfa)
Metabolic Disease Enzyme Replacement, Gaucher's Disease Intravenous Vial (Ea)
Last reviewed · Source: First Databank (FDB) drug data
Overview
Velaglucerase alfa is a biosynthetic (gene activation technology origin) form of human beta-glucocerebrosidase (glucosylceramidase), a lysosomal enzyme that catalyzes the hydrolysis of glucocerebroside into glucose and ceramide.
Velaglucerase alfa is a biosynthetic (gene activation technology origin) form of human beta-glucocerebrosidase (glucosylceramidase). Velaglucerase alfa is produced in a human fibroblast cell line in which the endogenous beta-glucocerebrosidase gene has been activated by targeting a potent enhancer/promoter to the gene locus. Unlike imiglucerase (a biosynthetic (recombinant DNA origin) form of human beta-glucocerebrosidase), velaglucerase alfa has the same amino acid sequence as the naturally occurring human enzyme.
Velaglucerase alfa is manufactured to contain predominantly high mannose-type N-linked glycan chains, which are specifically recognized and internalized via the mannose receptor present on the surface of macrophages (the cells that accumulate glucocerebroside in Gaucher disease). The high mannose-type N-linked glycan chains, similar to the exposed mannose residues present in imiglucerase, result in increased receptor specificity and improved activity of the exogenously administered enzyme.
As an enzyme replacement therapy, velaglucerase alfa is intended to replace the deficient endogenous enzyme glucocerebrosidase in patients with Gaucher disease. The enzymatic deficiency causes accumulation of glucocerebroside in various cells and organs throughout the body leading to clinical manifestations. Velaglucerase alfa increases the degradation of glucocerebroside to glucose and ceramide.
USES: Velaglucerase alfa is used to treat a certain rare genetic problem (Gaucher disease). Velaglucerase alfa replaces a certain natural substance (an enzyme called glucocerebrosidase) that is missing in people with Gaucher disease. This medication improves blood, bone, liver, and spleen problems caused by Gaucher disease.
Velaglucerase alfa does not correct the genetic problem, and treatment must be continued for life.
Generic name: velaglucerase alfa
Pronunciation: (VEL-a-GLOO-ser-ase Al-fa)
Drug class: Metabolic Disease Enzyme Replacement, Gaucher's Disease
Therapeutic class: Metabolic Disease Enzyme Replacement Agents
Reference product & biosimilars
No reference, biosimilar or interchangeable associated with this drug.
Today’s intelligence on Vpriv
Indications
The following indications are listed for Vpriv:
- Gaucher's Disease
Also known as: Cerebroside Lipidosis, Familial Splenic Anemia, Type 1 Gaucher Disease.
Dosing
The recommended initial dosage of velaglucerase alfa in adults and children 4-17 years of age with type 1 Gaucher disease is 60 units/kg every 2 weeks. The dosage of velaglucerase alfa can be adjusted based on achievement and maintenance of the patient's therapeutic goals. Dosages ranging from 15-60 units/kg every 2 weeks have been evaluated in clinical trials.
Patients receiving current treatment with imiglucerase can be switched to velaglucerase alfa at an equivalent dosage.
No dosing information is available for this tab.
| Drug label | Dosing type | Instructions |
|---|---|---|
| Vpriv 400 Units Vial | Maintenance | Infuse 15 unit/kg over 60 minute(s) by intravenous route every 2 weeks. |
No dosing information is available for this tab.
Drug interactions
There is no enhanced messaging for this drug.
There are no contraindication interactions for this drug.
There are no severe interactions for this drug.
There are no moderate interactions for this drug.
Contraindications
The manufacturer states that there are no known contraindications to the use of velaglucerase alfa.
There are no contraindication contraindications for this drug.
There are no severe contraindications for this drug.
- No disease contraindications
Adverse reactions
Adverse effects reported in 10% or more of patients receiving velaglucerase alfa include hypersensitivity reactions, headache, dizziness, pyrexia, abdominal pain, back pain, joint pain, asthenia/fatigue, and prolonged activated partial thromboplastin time (aPTT).
More Frequent
- Hypersensitivity drug reaction
Less Frequent
- Urticaria
Rare or Very Rare
- Anaphylaxis
- Dyspnea
- Hypertension
- Hypotension
- Vomiting
More Frequent
- Acute abdominal pain
- Arthralgia
- Back pain
- Dizziness
- Fatigue
- Fever
- General weakness
- Headache disorder
- Nausea
- Prolonged activated partial thromboplastin time
Less Frequent
- Bone pain
- Skin rash
- Tachycardia
Rare or Very Rare
- Chest discomfort
- Flushing
- Pruritus of skin
Precautions
No enhanced messaging available.
Safety and efficacy of velaglucerase alfa have not been established in children younger than 4 years of age.
Category B. (See Users Guide.) There are no adequate and well-controlled studies of velaglucerase alfa in pregnant women; the drug should be used during pregnancy only if clearly needed. Animal studies suggest no evidence of fetal harm.
It is not known whether velaglucerase alfa is distributed into milk. Caution is advised if the drug is administered to nursing women. The known benefits of breast-feeding should be considered along with the woman's clinical need for velaglucerase alfa and any potential adverse effects of the drug or disease on the infant.
Clinical response and adverse effects in geriatric patients appear to be similar to those of younger adults; in general, dosage should be selected with caution and the presence of any comorbid conditions should be considered in geriatric patients.
ICD codes
| Code | Type | Description |
|---|---|---|
| Gaucher's disease | ||
| E75.22 | ICD10CM | Gaucher disease |
ICD code information from First Databank (FDB).
Formulary status
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Last reviewed · Source: First Databank (FDB) drug data
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