Revcovi

Drug - Revcovi™ (elapegademase-lvlr) [Leadiant Biosciences, Inc.]

September 2019

Therapeutic area - Adenosine deaminase severe combined immunodeficiency disease (ADA-SCID)

Initial approval criteria

Patient has a diagnosis of severe combined immunodeficiency disease (SCID) with a definitive diagnosis of adenosine deaminase deficiency (ADA) as determined by one of the following:
- ADA catalytic activity (< 1% of normal) in hemolysates (in untransfused individuals) or in extracts of other cells (e.g., blood mononuclear cells, fibroblasts) OR
- Detection of pathogenic mutations in the ADA gene by molecular genetic testing AND
Patient has a marked elevation of the metabolite deoxyadenosine triphosphate (dATP) or total dAdo nucleotides (the sum of dAMP, dADP, and dATP) in erythrocytes AND
Patient is not a candidate for or has failed bone marrow transplantation (BMT) AND
Patient does not have severe thrombocytopenia (< 50,000/microL) AND
Baseline values for plasma ADA activity, red blood cell deoxyadenosine triphosphate (dATP), trough deoxyadenosine nucleotide (dAXP) levels, and/or total lymphocyte counts have been obtained

Patient continues to meet above criteria AND
Patient does not have unacceptable toxicity (e.g., severe injection site reactions/bleeding, severe thrombocytopenia) AND
Patient has adequate disease stability and/or response as indicated by 1 of the following:
- Increase in plasma ADA activity (target trough level ≥ 15 mmol/hour/L) OR
- Red blood cell dATP level decreased (target ≤ 0.005 to 0.015 mmol/L) OR
- Improvement in red blood cell dAXP levels (target trough level ≤ 0.02 mmol/L) OR
- Improvement in immune function with diminished frequency/complications of infection as evidenced in improvement in the ability to produce antibodies

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