Outcome of Surgical Interventions in Patients with Haemophilia A and B Treated with Extended Half-Life (EHL) Factor Concentrates in a Single Centre.

IF 2.4 4区 医学 Q2 HEMATOLOGY
Ines Vaide, Saskia Gottstein, Cornelia Kubicek-Hofmann, Ana Maria Orlovic, Robert Klamroth
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引用次数: 0

Abstract

The prevention of intraoperative bleeding in patients with haemophilia is the key to a successful surgical procedure. Daily life of patients with haemophilia A and B significantly improved with prophylaxis with extended half-life factor concentrates (EHL-FVIII and EHL-FIX). The aim of this study was to investigate the efficacy and safety of EHL factor concentrates during surgery.In a retrospective chart review all surgical interventions in our hospital in patients with haemophilia A and B treated with EHL-FVIII or EHL-FIX undergoing surgery between 2016 and 2022 were included. Patients with inhibitors against FVIII or FIX were excluded.A total of 88 surgical interventions (41 minor, 47 major) in 52 patients with haemophilia were performed. The interventions consisted of 70 surgeries in 42 patients with haemophilia A and 18 surgeries in 10 patients with haemophilia B. The replacement therapy during the surgeries was performed with four different EHL FVIII and three different EHL FIX concentrates. Bolus injections were performed directly before surgery and continued after surgery with variable intervals ranging from 8 to 48 hours. The median dose before major surgery was 32.31 IU/kg FVIII and 47.06 IU/kg FIX and before minor surgery was 27.78 IU/kg FVIII and 33.78 IU/kg factor IX. There were 11 complications including 4 bleeding complications during/after surgery. No thromboembolic event and no inhibitor against FVIII or FIX were detected during follow-up.The replacement therapy with EHL factor concentrates in surgical interventions in patients with haemophilia A and B is safe and effective.

单一中心延长半衰期(EHL)因子浓缩治疗血友病A和B患者手术干预的结果
预防血友病患者术中出血是手术成功的关键。延长半衰期因子浓缩物(EHL-FVIII和EHL-FIX)预防能显著改善血友病A和B患者的日常生活。本研究的目的是探讨EHL因子浓缩物在手术中的疗效和安全性。回顾性图表回顾了2016年至2022年期间在我院接受EHL-FVIII或EHL-FIX治疗的血友病a和B患者的所有手术干预措施。排除有FVIII或FIX抑制剂的患者。52例血友病患者共行88次手术干预(41次小手术,47次大手术)。干预措施包括42例A型血友病患者的70例手术和10例b型血友病患者的18例手术。手术期间使用4种不同的EHL FVIII和3种不同的EHL FIX浓缩液进行替代治疗。术前直接进行大剂量注射,术后以8至48小时不等的时间间隔继续注射。大手术前中位剂量为32.31 IU/kg FVIII和47.06 IU/kg FIX,小手术前中位剂量为27.78 IU/kg FVIII和33.78 IU/kg factor IX。合并症11例,其中术中/术后出血4例。随访期间未发现血栓栓塞事件,未发现FVIII或FIX抑制剂。在血友病A、B患者的手术干预中,以EHL因子为主的替代疗法是安全有效的。
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来源期刊
Hamostaseologie
Hamostaseologie HEMATOLOGY-
CiteScore
5.50
自引率
6.20%
发文量
62
审稿时长
6-12 weeks
期刊介绍: Hämostaseologie is an interdisciplinary specialist journal on the complex topics of haemorrhages and thromboembolism and is aimed not only at haematologists, but also at a wide range of specialists from clinic and practice. The readership consequently includes both specialists for internal medicine as well as for surgical diseases.
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