Perioperative replacement therapy in haemophilia B: An appeal to "B" more precise

Opti-Clot Study Grp, Jul-2018, In : Haemophilia. 24, 4, p. 611-618 8 p.

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  • Opti-Clot Study Grp

IntroductionHaemophilia 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.

AimTo 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.

MethodsIn this international multicentre study, haemophilia B patients with FIX

ResultsA total of 255 surgical procedures were performed in 118 patients (median age 40 years, median body weight 79kg). Sixty percent of FIX levels within 24hours of surgery were below target with a median difference of 0.22 IUmL(-1) [IQR 0.12-0.36]; while >6days after surgery, 59% of FIX levels were above target with a median difference of 0.19 IUmL(-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%).

ConclusionThis 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.

Original languageEnglish
Pages (from-to)611-618
Number of pages8
Issue number4
Publication statusPublished - Jul-2018


  • clotting factor concentrates, haemophilia B, haemostasis, individualized treatment, perioperative replacement therapy, surgical procedures, RECOMBINANT FACTOR-IX, FACTOR-VIII, A PATIENTS, PHARMACOKINETICS, PROPHYLAXIS, SURGERY, PATIENT, RISK, AGE

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