recombinant ADAMTS13-krhn

Indications

• prophylactic or on-demand enzyme replacement therapy for adult & pediatric patients with congenital thrombotic thrombocytopenic purpura (TTP)

Contraindications

• hypersensitivity reactions

Dosage

• prophylactic
  • 40 IU/kg every other week
  • intravenous infusion 2-4 mL/min
  • dosage may be given weekly if indicated by clinical response
• on demand
  • 40 IU/kg on day 1
  • 20 IU/kg on day 2
  • 15 IU/kg on day 3 & beyond until 2 days after the acure event has resolved

lyophilized powder in single-dose vials containing 500 or 1500 international units

Adverse effects

• antibodies to recombinant ADAMTS13 may develop
• > 5%
  • headache, migraine
  • nausea, vomiting, diarrhea, abdominal pain
  • upper respiratory tract infection, dizziness

Mechanism of action

• recombinant form of ADAMTS13

More general terms

• recombinant protein; chimer
• A disintegrin & metalloproteinase with thrombospondin type 1 motif 13