Perioperative replacement therapy in haemophilia B: An appeal to “B” more precise |
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| Authors: | H. C. A. M. Hazendonk T. Preijers R. Liesner P. Chowdary D. Hart D. Keeling M. H. E. Driessens B. A. P. Laros‐van Gorkom F. J. M. van der Meer K. Meijer K. Fijnvandraat F. W. G. Leebeek R. A. A. Mathôt P. W. Collins M. H. Cnossen the “OPTI‐CLOT” study group |
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| Affiliation: | 1. Department of Paediatric Haematology, Erasmus University Medical Centre ‐ Sophia Children's Hospital Rotterdam, Rotterdam, The Netherlands;2. Hospital Pharmacy ‐ Clinical Pharmacology, Academic Medical Centre Amsterdam, Amsterdam, The Netherlands;3. Great Ormond Street Haemophilia Centre, Great Ormond Street Hospital for Children NHS trust, London, UK;4. Katharine Dormandy Haemophilia Centre and Thrombosis Unit, Royal Free London NHS Foundation Trust, London, UK;5. Department of Haematology, The Royal London Hospital Barts Health NHS Trust, London, UK;6. Oxford Haemophilia and Thrombosis Centre, Oxford University Hospitals, Churchill Hospital, Oxford, UK;7. Netherlands Haemophilia Patient Society (NVHP), Nijkerk, The Netherlands;8. Department of Haematology, Radboud university medical centre, Nijmegen, The Netherlands;9. Department of Thrombosis and Haemostasis, Leiden University Medical Centre, Leiden, The Netherlands;10. Department of Haematology, University Medical Centre Groningen, University of Groningen, Groningen, The Netherlands;11. Department of Paediatric Haematology, Academic Medical Centre Amsterdam, Amsterdam, The Netherlands;12. Department of Haematology, Erasmus University Medical Centre Rotterdam, Rotterdam, The Netherlands;13. Arthur Bloom Haemophilia Centre, Institute of Infection and Immunity, School of Medicine, Cardiff University, Cardiff, UK |
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| Abstract: | Introduction Haemophilia B is caused by a deficiency of coagulation factor IX (FIX) and characterized by bleeding in muscles and joints. In the perioperative setting, patients are treated with FIX replacement therapy to secure haemostasis. Targeting of specified FIX levels is challenging and requires frequent monitoring and adjustment of therapy. Aim To evaluate perioperative management in haemophilia B, including monitoring of FIX infusions and observed FIX levels, whereby predictors of low and high FIX levels were assessed. Methods In this international multicentre study, haemophilia B patients with FIX < 0.05 IU mL?1 undergoing elective, minor or major surgical procedures between 2000 and 2015 were included. Data were collected on patient, surgical and treatment characteristics. Observed FIX levels were compared to target levels as recommended by guidelines. Results A total of 255 surgical procedures were performed in 118 patients (median age 40 years, median body weight 79 kg). Sixty percent of FIX levels within 24 hours of surgery were below target with a median difference of 0.22 IU mL?1 [IQR 0.12‐0.36]; while >6 days after surgery, 59% of FIX levels were above target with a median difference of 0.19 IU mL?1 [IQR 0.10‐0.39]. Clinically relevant bleeding complications (necessity of a second surgical intervention or red blood cell transfusion) occurred in 7 procedures (2.7%). Conclusion This study demonstrates that targeting of FIX levels in the perioperative setting is complex and suboptimal, but although this bleeding is minimal. Alternative dosing strategies taking patient and surgical characteristics as well as pharmacokinetic principles into account may help to optimize and individualize treatment. |
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| Keywords: | clotting factor concentrates haemophilia B haemostasis individualized treatment perioperative replacement therapy surgical procedures |
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