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
(2022) In Haemophilia 28(5). p.713-719- Abstract
Introduction: Perioperative dosing recommendations vary across Nordic haemophilia treatment centres (HTCs) for extended half-life (EHL) factor concentrates in haemophilia A/B (HA/HB) patients. Aim: To summarise Nordic real-world surgical experiences with EHL recombinant factor VIII/IX Fc (rFVIIIFc/rFIXFc) fusion proteins using retrospective data from clinical records at four HTCs in Finland, Sweden and Norway. Methods: Factor dosing and surgical outcomes were recorded from HA/HB patients who underwent surgery and were treated with rFVIIIFc/rFIXFc. Perioperative factor dosing regimens were clinician-determined based on local practises. Results: Twenty five surgeries were performed on 20 patients, all covered by bolus injections except... (More)
Introduction: Perioperative dosing recommendations vary across Nordic haemophilia treatment centres (HTCs) for extended half-life (EHL) factor concentrates in haemophilia A/B (HA/HB) patients. Aim: To summarise Nordic real-world surgical experiences with EHL recombinant factor VIII/IX Fc (rFVIIIFc/rFIXFc) fusion proteins using retrospective data from clinical records at four HTCs in Finland, Sweden and Norway. Methods: Factor dosing and surgical outcomes were recorded from HA/HB patients who underwent surgery and were treated with rFVIIIFc/rFIXFc. Perioperative factor dosing regimens were clinician-determined based on local practises. Results: Twenty five surgeries were performed on 20 patients, all covered by bolus injections except one minor HA surgery; eight minor surgeries were in paediatric patients. Median preoperative rFVIIIFc dose for major HA surgeries (n = 8) was 48 IU/kg (range: 35–57), with total consumption up to Day 14 of 427 IU/kg (196–568). For the two major HB surgeries (in one patient), preoperative rFIXFc doses were 50 IU/kg and 20 IU/kg; total consumption up to Day 14 was 130 IU/kg and 40 IU/kg. Median preoperative rFVIIIFc/rFIXFc bolus doses for minor HA (n = 10) and HB (n = 4) surgeries were 50 IU/kg (24–79) and 47 IU/kg (40–71), with total consumption up to Day 5 of 138 IU/kg (49–404) and 100 IU/kg (43–125), respectively. Intraoperative and postoperative haemostatic responses were rated as at least good/excellent for 24/25 surgeries, with bleeding episodes reported in only three surgeries. Conclusion: Nordic real-world experiences suggest that EHL products can be used safely and effectively for peri-operative haemostasis. Further research is required to develop local dosing guidelines for optimised treatment schedules.
(Less)
- author
- Lehtinen, Anna Elina ; Baghaei, Fariba ; Astermark, Jan LU and Holme, Pål André
- organization
- publishing date
- 2022
- type
- Contribution to journal
- publication status
- published
- subject
- keywords
- factor IX Fc fusion protein, factor VIII-Fc fusion protein, haemophilia A, haemophilia B, recombinant fusion proteins, surgical procedures
- in
- Haemophilia
- volume
- 28
- issue
- 5
- pages
- 713 - 719
- publisher
- Wiley-Blackwell
- external identifiers
-
- scopus:85132597465
- pmid:35575446
- ISSN
- 1351-8216
- DOI
- 10.1111/hae.14585
- language
- English
- LU publication?
- yes
- id
- 3b0a93e6-662d-46c0-a184-de1211c3460c
- date added to LUP
- 2022-09-06 15:27:32
- date last changed
- 2024-06-13 19:06:16
@article{3b0a93e6-662d-46c0-a184-de1211c3460c,
abstract = {{<p>Introduction: Perioperative dosing recommendations vary across Nordic haemophilia treatment centres (HTCs) for extended half-life (EHL) factor concentrates in haemophilia A/B (HA/HB) patients. Aim: To summarise Nordic real-world surgical experiences with EHL recombinant factor VIII/IX Fc (rFVIIIFc/rFIXFc) fusion proteins using retrospective data from clinical records at four HTCs in Finland, Sweden and Norway. Methods: Factor dosing and surgical outcomes were recorded from HA/HB patients who underwent surgery and were treated with rFVIIIFc/rFIXFc. Perioperative factor dosing regimens were clinician-determined based on local practises. Results: Twenty five surgeries were performed on 20 patients, all covered by bolus injections except one minor HA surgery; eight minor surgeries were in paediatric patients. Median preoperative rFVIIIFc dose for major HA surgeries (n = 8) was 48 IU/kg (range: 35–57), with total consumption up to Day 14 of 427 IU/kg (196–568). For the two major HB surgeries (in one patient), preoperative rFIXFc doses were 50 IU/kg and 20 IU/kg; total consumption up to Day 14 was 130 IU/kg and 40 IU/kg. Median preoperative rFVIIIFc/rFIXFc bolus doses for minor HA (n = 10) and HB (n = 4) surgeries were 50 IU/kg (24–79) and 47 IU/kg (40–71), with total consumption up to Day 5 of 138 IU/kg (49–404) and 100 IU/kg (43–125), respectively. Intraoperative and postoperative haemostatic responses were rated as at least good/excellent for 24/25 surgeries, with bleeding episodes reported in only three surgeries. Conclusion: Nordic real-world experiences suggest that EHL products can be used safely and effectively for peri-operative haemostasis. Further research is required to develop local dosing guidelines for optimised treatment schedules.</p>}},
author = {{Lehtinen, Anna Elina and Baghaei, Fariba and Astermark, Jan and Holme, Pål André}},
issn = {{1351-8216}},
keywords = {{factor IX Fc fusion protein; factor VIII-Fc fusion protein; haemophilia A; haemophilia B; recombinant fusion proteins; surgical procedures}},
language = {{eng}},
number = {{5}},
pages = {{713--719}},
publisher = {{Wiley-Blackwell}},
series = {{Haemophilia}},
title = {{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}},
url = {{http://dx.doi.org/10.1111/hae.14585}},
doi = {{10.1111/hae.14585}},
volume = {{28}},
year = {{2022}},
}