Continuous infusion factor replacement in haemophilia B during and after cardiac surgery: the better choice?

BMJ Case Rep. 2020 Nov 30;13(11):e235859. doi: 10.1136/bcr-2020-235859.

Abstract

A 57-year-old man with mild haemophilia B was admitted for coronary artery bypass graft surgery. His factor IX (FIX) activity was 15% on admission. Our goal was to maintain his FIX activity at 80%-100% for post-op days (PODs) 0-3, and at 60%-80% for PODs 4-14. Preoperatively, the patient was given recombinant FIX (rFIX) bolus using the formula:Dosage needed=%(desired FIX level-current level of FIX)×weight (kg)×1.3.This increased his activity to 100%. One IU of rFIX increased FIX activity by 0.8%; the half-life of rFIX is 18-24 hours. The rFIX infusion was started intraoperatively and continued after surgery to maintain target FIX activity. He was discharged on POD 9 on rFIX bolus dosing of 5000 IU every 12 hours for an additional 5 days. Using continuous factor infusion, we managed to decrease the amount rFIX used by >60% while maintaining steady state FIX activity level.

Keywords: haematology (drugs and medicines); malignant and benign haematology.

Publication types

  • Case Reports

MeSH terms

  • Coronary Artery Bypass*
  • Factor IX / administration & dosage*
  • Factor IX / metabolism
  • Hemophilia B / blood
  • Hemophilia B / complications
  • Hemophilia B / drug therapy*
  • Humans
  • Infusions, Intravenous
  • Male
  • Middle Aged
  • Recombinant Proteins / administration & dosage

Substances

  • Recombinant Proteins
  • Factor IX