Elelyso (taliglucerase alfa)
Metabolic Disease Enzyme Replacement, Gaucher's Disease Intravenous Vial (Ea)
Last reviewed · Source: First Databank (FDB) drug data
Overview
Taliglucerase alfa is a biosynthetic (recombinant DNA origin) form of human glucocerebrosidase, a lysosomal enzyme that catalyzes the hydrolysis of glucocerebroside to glucose and ceramide.
Taliglucerase alfa is a biosynthetic (recombinant DNA origin) form of human beta-glucocerebrosidase (glucosylceramidase). The drug is produced in genetically modified carrot plant root cells cultured in a disposable bioreactor system. Taliglucerase alfa differs from naturally occurring human glucocerebrosidase by 2 amino acids at the N-terminal and up to 7 amino acids at the C-terminal.
Purified taliglucerase alfa is a monomeric glycoprotein containing 4 N-linked mannose-terminated glycosylation sites, which are specifically recognized by mannose receptors present on the surface of macrophages (the cells that accumulate glucocerebroside in Gaucher disease).
As an enzyme replacement therapy, taliglucerase alfa is intended to replace the deficient lysosomal 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. Taliglucerase alfa increases the degradation of glucocerebroside to glucose and ceramide.
In clinical studies, treatment with the drug resulted in reduced spleen and liver volumes and improved anemia and thrombocytopenia. (See Uses.)
Potency of taliglucerase alfa is expressed in units. Each unit of taliglucerase alfa is defined as the amount of enzyme that catalyzes the hydrolysis of 1 mcmol of the synthetic substrate nitrophenyl-beta-D-glucopyranoside (pNP-Glc) in 1 minute at 37degreesC.
USES: Taliglucerase alfa is used to treat a certain rare genetic problem (Gaucher disease). Taliglucerase 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.
Taliglucerase alfa does not correct the genetic problem, and treatment must be continued for life.
Generic name: taliglucerase alfa
Pronunciation: (TAL-i-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 Elelyso
Indications
The following indications are listed for Elelyso:
- Gaucher's Disease
Also known as: Cerebroside Lipidosis, Familial Splenic Anemia, Type 1 Gaucher Disease.
Dosing
The recommended dosage of taliglucerase alfa in treatment-naive adults and pediatric patients 4 years of age and older with type 1 Gaucher disease is 60 units/kg every other week. Dosage can be adjusted based on achievement and maintenance of the patient's therapeutic goals.
Patients currently receiving imiglucerase (at a stable dosage) can be switched to taliglucerase alfa at the same dosage.
No dosing information is available for this tab.
| Drug label | Dosing type | Instructions |
|---|---|---|
| Elelyso 200 Units Vial | Maintenance | Infuse 60 unit/kg over 60-120 minute(s) by intravenous route every 2 weeks. |
No dosing information is available for this tab.
Drug interactions
Formal drug interaction studies have not been performed to date.
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 taliglucerase 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 taliglucerase alfa include infusion reactions, upper respiratory tract infection or nasopharyngitis, pharyngitis or throat infection, headache, arthralgia, influenza, urinary tract infection or pyelonephritis, back pain, and pain in extremities.
Rare or Very Rare
- Anaphylaxis
- Angioedema
- Hypotension
More Frequent
- Acute abdominal pain
- Arthralgia
- Back pain
- Dizziness
- Flushing
- Headache disorder
- Hypersensitivity drug reaction
- Nausea
- Pain in extremities
- Pruritus of skin
- Urticaria
- Vomiting
Less Frequent
- Chest pain
- Diarrhea
- Erythema
- Fatigue
- Skin rash
Rare or Very Rare
- Chest tightness
- Wheezing
Precautions
No enhanced messaging available.
Use of taliglucerase alfa in pediatric patients 4 years of age or older is supported principally by adequate and well-controlled studies of the drug in adults. In general, adverse effects are similar between adults and pediatric patients with the exception of vomiting, which has been reported with higher frequency in pediatric patients.
There is insufficient experience with taliglucerase alfa in pregnant women; animal studies have not suggested any evidence of adverse embryofetal effects.
It is not known whether taliglucerase alfa is distributed into milk. The known benefits of breast-feeding should be considered along with the woman's clinical need for taliglucerase alfa and any potential adverse effects of the drug or disease on the infant.
Clinical studies of taliglucerase alfa did not include sufficient numbers of patients 65 years of age and older to determine whether geriatric patients respond differently than younger adults. In clinical studies, only 8 patients 65 years of age and older received taliglucerase alfa.
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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