Dosing & Uses
Adenosine Deaminase Deficiency
1/22/2019: Discontinuation is due to the permanent shortage of the active ingredient
Based on the demand in mid-January 2019, the inventory is estimated to runout by late-March 2019
Adenosine Deaminase Deficiency
1/22/2019: Discontinuation is due to the permanent shortage of the active ingredient
Based on the demand in mid-January 2019, the inventory is estimated to runout by late-March 2019
Interactions
Interaction Checker
No Results
Contraindicated
Serious - Use Alternative
Significant - Monitor Closely
Minor
Contraindicated (0)
Serious - Use Alternative (1)
- pentostatin
pentostatin decreases effects of pegademase by Other (see comment). Avoid or Use Alternate Drug. Comment: Pentostatin is a potent inhibitor of adenosine deaminase (pegademase).
Monitor Closely (2)
- pegloticase
pegloticase will decrease the level or effect of pegademase by Other (see comment). Use Caution/Monitor. According to the manufacturer, potential for anti-PEG antibody development that may bind to other pegylated drugs; clinical significance unknown
- pegvaliase
pegvaliase, pegademase. Other (see comment). Use Caution/Monitor. Comment: The majority of patients treated with pegvaliase develop anti-polyethylene glycol (PEG) IgM and IgG antibodies. Risk of coadministration with different PEGylated products is unknown. There is a case report of anaphylaxis following a medroxyprogesterone acetate injectable suspension that contained PEG 3350. Carefully read all drug labels, including OTC drugs to check contents for PEG. Note: Unable to include an exhaustive product list for this interaction.
Minor (0)
Adverse Effects
Frequency Not Defined
Hematologic events: Hemolytic anemia, autoimmune hemolytic anemia, thrombocythemia, thrombocytopenia and autoimmune thrombocytopenia
Dermatological events: Injection site erythema, urticaria
Lymphomas
Headache
Warnings
Contraindications
Use as a preparatory/support therapy for bone marrow transplantation
Severe thrombocytopenia
Cautions
Use caution in patients with thrombocytopenia; contraindicated in patients with severe thrombocytopenia
Monitor plasma ADA activity & red blood cell dATP levels
Development of antibodies reported, which may result in rapid clearance; adjust dose as necessary in patients developing antibodies
Risk for infection increases in patients that are not able to maintain adequate levels of plasma ADA
Not a substitute for bone marrow transplant
Pregnancy & Lactation
Pregnancy Category: C
Lactation: Not known if distributed into breast milk, use caution
Pregnancy Categories
A: Generally acceptable. Controlled studies in pregnant women show no evidence of fetal risk.
B: May be acceptable. Either animal studies show no risk but human studies not available or animal studies showed minor risks and human studies done and showed no risk. C: Use with caution if benefits outweigh risks. Animal studies show risk and human studies not available or neither animal nor human studies done. D: Use in LIFE-THREATENING emergencies when no safer drug available. Positive evidence of human fetal risk. X: Do not use in pregnancy. Risks involved outweigh potential benefits. Safer alternatives exist. NA: Information not available.Pharmacology
Mechanism of Action
Replaces adenosing deaminase deficiency; the enzyme that catalyzes the deamination of deoxyadenosine and adenosine; this results in combined immunodeficiency disease with profound cellular and humoral immunity
Pharmacokinetics
Half-life: 3-6 days
Peak plasma time: 2-3 days
Absorption: Rapid
