Single Arm, Open-Label, Multicenter, Registry Study of Revcovi (Elapegademase-lvlr) Treatment in ADA-SCID Patients Requiring Enzyme Replacement Therapy

elapegademase-lvlr

Patients transitioning from Adagen: For patients currently receiving Adagen at ≤ 30 U/kg/wk or an unknown Adagen dose, the suggested dosage of Revcovi is 0.2 mg/kg/wk IM. For patients currently receiving Adagen at \> 30 U/kg/wk the suggested equivalent Revcovi dosage (mg/kg/wk) is the Adagen dosage in U/kg/wk divided by 150. At the investigator's discretion, the total weekly dose may be divided and administered in multiple IM injections, increased by 0.033 mg/kg/wk if trough ADA activity is \< 30 mmol/hr/L, dAXP is \> 0.02 mmol/L, and/or the immune reconstitution is inadequate. Adagen-naïve patients: The suggested starting Revcovi dosage is 0.2 mg/kg twice weekly IM based on ideal body weight, for a minimum of 12 to 24 weeks until immune reconstitution is achieved. At the investigator's discretion, the dosage may be gradually increased to maintain trough ADA activity \> 30 mmol/hr/L, dAXP \< 0.02 mmol/L, and/or to maintain adequate immune reconstitution.

Deoxyadenosine nucleotides (dAXP) activity

Month 24

ADA activity

Month 24