Miscellaneous immune disorders:

Indications for REVCOVI:

Treatment of adenosine deaminase severe combined immune deficiency (ADA-SCID).

Adults and Children:

Individualize. Adjust dose based on trough ADA activity, trough dAXP levels, and/or patient's clinical status (see full labeling). Give as IM inj; rotate sites. Transitioning from Adagen (weekly Adagen dose ≤30 U/kg or unknown): initially 0.2mg/kg once weekly; (weekly Adagen dose >30 U/kg): calculate dose using conversion formula (Revcovi dose [mg/kg] = Adagen dose [U/kg]/150). May increase subsequent doses in 0.033mg/kg increments weekly based on appropriate trough levels. Adagen-naive: initially 0.4mg/kg weekly (based on ideal body weight) divided into 2 doses (0.2mg/kg twice weekly) for at least 12–24 weeks until immune reconstitution achieved. May gradually decrease dose to maintain appropriate trough levels and/or adequate immune reconstitution.

Warnings/Precautions:

Determine trough plasma ADA activity and trough dATP level prior to first administration. After initiation, measure trough plasma ADA activity (pre-injection) every 2 weeks (Adagen-naive) or 4 weeks (Adagen-experienced), during first 8–12 weeks of treatment, then every 3–6 months thereafter. Adjust dose if persistent fall in trough plasma ADA activity due to antibodies development. Monitor trough erythrocyte dAXP levels at least twice a year after 2 months of treatment. Monitor lymphocytes every 4–8 weeks for up to 1 year, and every 3–6 months thereafter (Adagen-naive), or every 3–6 months (others). Thrombocytopenia; do not initiate if severe. Protect immune deficient patients from infections until immune function improved. Elderly (≥65yrs): not studied. Pregnancy: monitor closely. Nursing mothers.

Pharmacologic Class:

Recombinant adenosine deaminase.

Adverse Reactions:

Cough, vomiting; possible immunogenicity.

Generic Availability:

NO

How Supplied:

Single-dose vial—1