Pegademase Bovine Brand Name– Adagen
What is Pegademase Bovine
Pegademase bovine is parenteral enzyme replacement therapy for patients with severe combined immunodeficiency disease (SCID) associated with adenosine deaminase (ADA) deficiency.
It is a conjugate of multiple strands of monomethoxypolyethylene glycol attached to ADA derived from bovine intestine.
SCID associated with ADA deficiency is a rare, inherited disorder that is often fatal. Patients with this disorder experience an accumulation of adenosine and 2′-deoxyadenosine, which causes metabolic abnormalities that are directly toxic to lymphocytes.
SCID can be cured only by bone marrow transplantation. Red blood cell transfusions have also been used as a method of non-specific ADA replacement; however, there is risk of adverse events associated with frequent transfusions, such as iron overload and infection transmission.
Pegademase bovine is specific ADA enzyme replacement and will not benefit patients with immunodeficiency due to other causes.
Adherence to a strict treatment schedule of pegademase bovine therapy, including careful monitoring of plasma ADA activity and red blood cell dATP levels, has been shown to eliminate the toxic metabolites caused by ADA deficiency.
Immune function, including the ability to produce antibodies, generally improves after 2—6 months of therapy, and may continue to improve over a longer period.
Pegademase bovine (Adagen®) was approved by the FDA for the treatment of severe combined immunodeficiency disease caused by adenosine deaminase deficiency on March 21, 1990.
Indications
- adenosine deaminase (ADA) deficiency
For the treatment of severe combined immunodeficiency disease (SCID) due to adenosine deaminase (ADA) deficiency
Side Effects
- antibody formation
- erythema
- headache
- hemolytic anemia
- injection site reaction
- new primary malignancy
- thrombocytopenia
- thrombocytosis
- urticaria
During clinical trials examining pegademase bovine, headache was reported in 1 patient and injection site reaction (i.e. pain) was reported in 2 patients
Monitoring Parameters
- laboratory monitoring not necessary
Contraindications
- breast-feeding
- coagulopathy
- infection
- pregnancy
- thrombocytopenia
Interactions
There are no drug interactions associated with Pegademase Bovine products.
