Single centre, real‐world experience of perioperative rFIXFc use in adult patients with haemophilia B undergoing major and minor surgery

Abstract

Introduction: Recombinant factor IX fusion protein concentrate (rFIXFc) is increasingly used for prophylaxis in people with haemophilia B (PWHB), but experience in the perioperative setting is limited. Aims:To evaluate real-world perioperative factor usage, bleeding and complications in PWHB (≥18 years) who received rFIXFc for surgical haemostasis and to describe the treatment regimens used.Methods: Single centre, retrospective review of all PWHB who underwent a major or minor surgical procedure between June 2017 and July 2020 and received rFIXFc perioperatively for maintenance of surgical haemostasis.Results: A total of 56 PWHB (45 male and 11 female), including people with mild (n = 32), moderate (n = 4) and severe (n = 20) haemophilia B, underwent 11 major and 131 minor procedures with rFIXFc for surgical haemostasis. Haemostasis was rated as excellent (9/11) or good (2/11) in all major procedures. Median total rFIXFc consumption for orthopaedic surgeries was 972 IU/kg (range 812-1031 IU/kg) and for other major (non-orthopaedic) surgeries was 323 IU/kg (range 167-760 IU/kg). The median number of perioperative rFIXFc infusions was 19 (range 17-26) for orthopaedic surgery and 7 (range 5-17) for other major surgeries. The number of infusions in the postoperative period was determined by procedure and patient factors. Complications included bowel ileus and wound infection. Most minor procedures were managed with single infusion of rFIXFc, with no bleeding complications in 95% of minor procedures. There were no thromboembolic events or inhibitor formation. Conclusion:This unique data provides real-world evidence that rFIXFc is safe and effective in achieving haemostasis in PWHB undergoing surgery.

Managing invasive procedures in haemophilia patients with limited resources, extended half‐life concentrates or non‐replacement therapies in 2022

“…Studies performed with the different EHL-rFIX have demonstrated that these products are effective and well tolerated for the prevention and treatment of bleeds during major orthopaedic and nonorthopaedic surgeries as well as for other minor procedure. [20][21][22][23] The efficacy and safety results were consistent with those previously reported for unmodified FIX. No FIX inhibitor, thromboembolic events or clinically significant safety issues were detected and reported in published studies.…”

“…The safety, efficacy and consumption of all EHL in limited number of patients undergoing surgery and invasive procedures have recently been evaluated in several pivotal trials including pegylated EHL-rFVIII, [16][17][18] Fc-Fused EHL-rFVIII, 19 Fc-fused EHL-rFIX, 20,21 albuminfused EHL-rFIX 22 and pegylated EHL-rIX. 23 As reviewed hereafter, the use of EHL concentrates impacts on the perioperative haemostatic management of surgery and invasive procedures compared SHL-FVIII/FIX.…”

“…The safety, efficacy and consumption of all EHL in limited number of patients undergoing surgery and invasive procedures have recently been evaluated in several pivotal trials including pegylated EHL‐rFVIII, 16–18 Fc‐Fused EHL‐rFVIII, 19 Fc‐fused EHL‐rFIX, 20,21 albumin‐fused EHL‐rFIX 22 and pegylated EHL‐rIX 23 …”

Surgical outcomes in patients with haemophilia A or B receiving extended half‐life recombinant factor VIII and IX Fc fusion proteins: Real‐world experience in the Nordic countries

“…Real‐world treatment of patients undergoing surgery with rFVIIIFc or rFIXFc in countries with similar levels of available resources as the Nordic countries has been reported in several case reports and studies 16–19,21–24,36 . Most of these report good surgical outcomes; however, only a few reports provide sufficient detail to compare dosing strategies.…”

“…Real-world treatment of patients undergoing surgery with rFVII-IFc or rFIXFc in countries with similar levels of available resources as the Nordic countries has been reported in several case reports and studies. [16][17][18][19][21][22][23][24]36 Most of these report good surgical outcomes; however, only a few reports provide sufficient detail to compare dosing strategies. In a case report from the United Kingdom in which an adult patient with severe HA underwent a major surgery, the reported preoperative rFVIIIFc dose of 64 IU/kg was higher than the range for major surgery cases in HA reported in our results; total rFVIIIFc consumption up to Day 14 of around 279 IU/kg was lower than all but two of the major HA cases reported here.…”