Elapegademase

Elapegademase Brand Name– REVCOVI

What is Elapegademase

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

It is a conjugate of multiple strands of monomethoxypolyethylene glycol attached to ADA derived from bovine intestine.

SCID associated with ADA deficiency is a rare, inherited disorder that is often fatal. Patients with this disorder experience an accumulation of adenosine and 2′-deoxyadenosine, which causes metabolic abnormalities that are directly toxic to lymphocytes.

Patients with SCID have extreme susceptibility to infection, which can lead to death in infancy if immunologic reconstitution cannot be achieved. The majority of patients affected also have significant lymphopenia.

The frequency of SCID has been estimated to range from 1 in 50,000 to 1 in 500,00 births. SCID can only be cured by bone marrow transplantation.

Elapegademase is a specific ADA enzyme replacement and will not benefit patients with immunodeficiency due to other causes.

Adherence to a strict treatment schedule of elapegademase therapy, including careful monitoring of trough plasma ADA activity, trough deoxyadenosine nucleotide (dAXP), and/or total lymphocyte counts, has been shown to increase plasma or serum ADA activity and lymphocyte counts and eliminate the toxic metabolites caused by ADA deficiency.

Immune function, including the ability to produce antibodies, generally improves after 2 to 6 months of therapy and may continue to improve over a longer period.

Indications

• adenosine deaminase (ADA) deficiency

For the treatment of severe combined immunodeficiency disease (SCID) due to adenosine deaminase (ADA) deficiency

for patients not currently receiving pegademase bovine

Side Effects

1. abdominal pain
2. antibody formation
3. arthralgia
4. asthenia
5. candidiasis
6. conjunctivitis
7. cough
8. diarrhea
9. epistaxis
10. erythema
11. fatigue
12. hemolytic anemia
13. hemoptysis
14. infection
15. influenza
16. lymphadenopathy
17. migraine
18. nausea
19. nephrolithiasis
20. neutropenia
21. new primary malignancy
22. rash
23. seizures
24. thrombocytopenia
25. thrombocytosis
26. urticaria
27. vomiting

Monitoring Parameters

• laboratory monitoring not necessary

Contraindications

• breast-feeding
• coagulopathy
• infection
• pregnancy
• thrombocytopenia

Interactions

There are no drug interactions associated with Elapegademase products.