Elapegademase

What it is and how it works

Mechanism of Action SCID associated with a deficiency of ADA enzyme is a rare, inherited, and often fatal disease. ADA enzyme is involved in purine metabolism, catalyzing the irreversible hydrolytic deamination of adenosine or deoxyadenosine to inosine or deoxyinosine, respectively, as well as several naturally occurring methylated adenosine compounds. Maintaining a low level of 2'-deoxyadenosine and adenosine is crucial for proper number and function of immune cells as well as decreasing the frequency of opportunistic infections. Elevated adenosine levels, as occurring in ADA deficiency, contribute to apoptosis and a block in the differentiation of thymocytes, causing severe T‑lymphopenia.

Indications

is indicated for the treatment of adenosine deaminase severe combined immune deficiency (ADA-SCID) in pediatric and adult patients. REVCOVI is a recombinant adenosine deaminase indicated for the treatment of adenosine deaminase severe combined immune deficiency (ADA-SCID) in pediatric and adult patients.

Dosage and administration (summary)

Patients transitioning from Adagen to REVCOVI : The starting dose of REVCOVI is 0.2 mg/kg weekly, intramuscularly. See Full Prescribing Information (FPI) for conversion formula from Adagen to REVCOVI. Adagen-naïve patients : The starting dose of REVCOVI is 0.4 mg/kg weekly based on ideal body weight or actual weight whichever is greater, divided into two doses (0.2 mg/kg twice a week), intramuscularly. For complete information, maintenance dosing and therapeutic monitoring, see FPI. ( 2.1 , 2.3 ) REVCOVI is for intramuscular injection only. See FPI for administration instructions.

Contraindications

None. None

Warnings and precautions

Injection Site Bleeding in Patients with Thrombocytopenia: Increased risk of local bleeding in patients with thrombocytopenia; should not be used if thrombocytopenia is severe. Delay in Improvement of Immune Function: Protect immune deficient patients from infections until improvement in immune function. Injection Site Bleeding in Patients with Thrombocytopenia Since REVCOVI is administered by IM injection, it should be used with caution in patients with thrombocytopenia and should not be used if thrombocytopenia is severe.

Pregnancy

Pregnancy Risk Summary Adequate and well-controlled studies with REVCOVI have not been conducted in pregnant women to inform a drug-associated risk. Animal reproduction studies have not been conducted with REVCOVI. It is not known whether REVCOVI can cause fetal harm when administered to a pregnant woman or can affect reproduction capacity.

Storage and supply

(elapegademase-lvlr) injection, 2.4 mg/1.5 mL (1.6 mg/mL), is a sterile, preservative free, clear, colorless solution for intramuscular use available as one single-dose vial per carton (NDC 10122-502-01). The vial stopper is not made with natural rubber latex. Single-dose vial; do not re-use the vial. Discard unused portions.

Source: Elapegademase-Lvlr (Revcovi) — US FDA prescribing information, 2026-05-31, DailyMed. Summarised and shortened; the full label governs. Iranian registration, indications and labelling may differ.