Lamzede (velmanase alfa-tycv)
Metabolic Disease Enzyme Replacement, Alpha-Mannosidosis Intravenous Vial (Ea)
Last reviewed · Source: First Databank (FDB) drug data
Overview
Velmanase alfa-tycv is a recombinant preparation of human lysosomal alpha-mannosidase.
Velmanase alfa-tycv is a recombinant form of the human alpha-mannosidase enzyme. The drug functions as an enzyme replacement therapy for the deficient endogenous alpha-mannosidase enzyme in patients with alpha-mannosidosis. Velmanase alfa-tycv is produced by recombinant DNA technology in Chinese Hamster Ovary cells; the amino acid sequence of the monomeric protein is identical to the naturally occurring human alpha-mannosidase enzyme.
Alpha-mannosidosis is a lysosomal storage disorder caused by reduced activity of the enzyme alpha-D-mannosidase; deficiency of this enzyme leads to accumulation of mannose-rich oligosaccharides. Velmanase alfa-tycv provides an exogenous source of alpha-D-mannosidase. The drug is internalized via binding to the mannose-6-phosphate receptor on the cell surface and transported into lysosomes where it is thought to exert its enzyme activity.
Pharmacokinetic data for velmanase alfa-tycv are limited. The metabolic pathway has not been characterized, but is expected to involve catabolic pathways metabolizing the drug into small peptides. The mean terminal half-life of velmanase alfa-tycv is 33.6 hours in patients with alpha-mannosidosis.
Generic name: velmanase alfa-tycv
Drug class: Metabolic Disease Enzyme Replacement, Alpha-Mannosidosis
Therapeutic class: Metabolic Disease Enzyme Replacement Agents
Reference product & biosimilars
No reference, biosimilar or interchangeable associated with this drug.
Today’s intelligence on Lamzede
Indications
The following indications are listed for Lamzede:
- Alpha-Mannosidosis
Also known as: Alpha-D-Mannosidase Deficiency, Alpha-Mannosidase Deficiency.
Dosing
In the event of a mild to moderate hypersensitivity reaction or a mild to moderate infusion-associated reaction (IAR), consider temporarily holding the infusion for 15-30 minutes, slowing the infusion rate to 25-50% of the recommended rate, and initiating appropriate medical treatment. If symptoms persist after slowing the infusion, stop the infusion and monitor the patient. If symptoms continue to persist, consider re-initiating the infusion within 7-14 days at 25-50% of the recommended rate with appropriate pretreatment. If symptoms subside after holding or slowing the infusion, resume the infusion at 25-50% of the recommended rate. If tolerated, increase the infusion rate by 25% of the recommended rate until the recommended infusion rate is reached. Closely monitor the patient.
In the event of a severe hypersensitivity reaction, including anaphylaxis, or severe IAR, immediately discontinue the infusion and initiate appropriate medical treatment.
No dosing information is available for this tab.
| Drug label | Dosing type | Instructions |
|---|---|---|
| Lamzede 10 Mg Vial | Maintenance | Infuse 1 mg/kg by intravenous route once weekly. |
No dosing information is available for this tab.
Drug interactions
No formal drug interaction studies have been performed to date. Velmanase alfa-tycv is not a substrate or inhibitor/inducer of major cytochrome P-450 (CYP) enzymes, or a substrate or inhibitor of major transporters.
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
*None
There are no contraindication contraindications for this drug.
There are no severe contraindications for this drug.
- No disease contraindications
Adverse reactions
Most common adverse reactions (incidence >20%) are hypersensitivity reactions (including anaphylaxis), nasopharyngitis, pyrexia, headache, and arthralgia.
More Frequent
- Anaphylaxis
- Hypersensitivity drug reaction
Less Frequent
- Gastroenteritis
- Influenza
Rare or Very Rare
- Angioedema
- Hearing loss
- Hypotension
- IgA vasculitis
- Seizure disorder
More Frequent
- Fever
- Headache disorder
- Pharyngitis
Less Frequent
- Arthralgia
- Back pain
- Infection of ear
- Ocular itching
- Syncope
- Tonsillitis
- Toothache
- Urinary tract infection
Rare or Very Rare
- Agitation
- Anorexia
- Arthritis
- Ataxia
- Drowsy
- Drug-induced psychosis
- Eye tearing
- Fecal incontinence
- General weakness
- Nervousness
- Odynophagia
- Palpitations
- Pharyngeal edema
- Tachycardia
- Wheezing
Precautions
No enhanced messaging available.
The safety and effectiveness of velmanase alfa-tycv have been established in pediatric patients. Pediatric patients treated with velmanase alfa-tycv experienced a higher incidence of hypersensitivity reactions compared to adults.
There is no adequate data regarding use of velmanase alfa-tycv in pregnant women. Based on findings from animal reproductive studies, velmanase alfa-tycv may cause embryo-fetal harm when administered to pregnant females.
The decision to continue or discontinue velmanase alfa-tycv during pregnancy should consider the patient's need for the drug, the potential drug-related risk to the fetus, and the potential adverse outcomes from untreated disease.
It is not known whether velmanase alfa-tycv or its metabolite is distributed into human milk, or if the drug has any effects on the breastfed infant or on milk production. The developmental and health benefits of breastfeeding should be considered along with the mother's clinical need for velmanase alfa-tycv and any potential adverse effects on the breastfed infant from the drug or underlying maternal condition.
Alpha-mannosidosis is largely a disease of pediatric and young adult patients. Safety and efficacy of velmanase alfa-tycv have not been established in geriatric patients 65 years of age and older.
ICD codes
| Code | Type | Description |
|---|---|---|
| Alpha-mannosidosis | ||
| E77.1 | ICD10CM | Defects in glycoprotein degradation |
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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