ADAMTS13, RECOMBINANT-KRHN INJ,LYPHL
Clinical Criteria Summary
Indication & Patient Population
- Prophylactic or on demand enzyme replacement therapy (ERT) in adult and pediatric patients with congenital thrombotic thrombocytopenic purpura (cTTP).
Dosage & Administration
- Prophylactic: 40 IU/kg once every other week at 2-4 mL per minute.
- On-demand: Day 1: 40 IU/kg; Day 2: 20 IU/kg; Day 3 and beyond: 15 IU/kg until 2 days after acute event is resolved, administered at 2-4 mL per minute.
- For on demand replacement: administer daily until 2 days after acute event is resolved.
Safety & Contraindications
- Contraindicated in patients with life threatening or hypersensitivity reactions to ADAMTS13 or any of its components.
- Monitor for hypersensitivity and immunogenicity (may develop neutralizing antibodies).
- Use during pregnancy and lactation is left to clinical judgment due to lack of data.
Place in Therapy & Alternatives
- For cTTP in remission, administer ADAMTS13 prophylaxis if it is available.
- If ADAMTS13 is not available, discuss use of Fresh Frozen Plasma in a shared decision-making process.
- Revisit formulary decision for ADAMTS13 as more data becomes available for acquired/autoimmune thrombotic thrombocytopenic purpura.