Adenosine deaminase severe combined immune deficiency (ADA-SCID).
Replaces adenosine deaminase. Without this enzyme, adenosine accumulates, which leads to apoptosis, lymphopenia, and opportunistic infections.
Increased lymphocyte concentrations.
Metabolism and Excretion: Unknown.
TIME/ACTION PROFILE (plasma concentrations)
Use Cautiously in:
* CAPITALS indicate life-threatening.
Underline indicate most frequent.
IM (Adults and Children): Transitioning from pegademase bovine weekly dose ≤30 units/kg or unknown– 0.2 mg/kg once weekly; may ↑ dose by increments of 0.033 mg/kg if trough adenosine deaminase activity <30 mmol/hr/L, trough deoxyadenosine nucleotides >0.02 mmol/L, and immune reconstitution not achieved. Transitioning from pegademase bovine weekly dose >30 units/kg– elapegademase dose (in mg/kg) (given once weekly) = pegademase bovine dose (in units/kg) /150; may ↑ dose by increments of 0.033 mg/kg if trough adenosine deaminase activity <30 mmol/hr/L, trough deoxyadenosine nucleotides >0.02 mmol/L, and immune reconstitution not achieved. Pegademase bovine-naïve– 0.2 mg/kg (based on ideal body weight or actual body weight, whichever is greater) twice weekly for ≥12–24 wk until immune reconstitution achieved, then ↓ dose to maintain trough adenosine deaminase activity >30 mmol/hr/L, trough deoxyadenosine nucleotides <0.02 mmol/L, and immune reconstitution.
Solution for injection: 1.6 mg/mL
Lab Test Considerations:
Monitor trough plasma ADA activity, trough dAXP levels, and/or total lymphocyte counts every 2 wk for patients new to therapy and every 4 wk for patient previously receiving therapy, during 1st 8–12 wk of therapy for up to 1 yr and every 3–6 mo thereafter. Monitor more frequent if therapy was interrupted or if an enhanced rate of clearance of plasma ADA activity develops. Collect blood samples for the analysis of trough plasma ADA activity and trough dAXP level prior to the first administration of elapegademase for the week. Target trough plasma ADA activity at least 30 mmol/hr/L. Monitor trough erythrocyte dAXP levels at least twice yearly and maintain at <0.02 mmol/L.
Trough erythrocyte dAXP levels <0.02 mmol/L.
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