Department of Orthopaedic Surgery, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Shuai Fu Yuan 1#, Dongcheng District, Beijing, 100730, China.
Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, 100730, China.
J Orthop Surg Res. 2024 Jun 16;19(1):358. doi: 10.1186/s13018-024-04843-4.
Haemophilia A (HA) is an X-linked recessive bleeding disorder caused by lack or deficiency of coagulation factor VIII.
The aim of this study is to determine the incidence and treatment-related risk factors of inhibitor development after intensive FVIII replacement for major orthopaedic surgery in previous treated persons with HA.
A total of 151 HA who underwent 221 major orthopaedic surgical procedures after intensive FVIII treatment were reviewed. The results of inhibitor tests were collected. Potential clinical risk factors for inhibitor development were analyzed.
111 people were diagnosed with severe HA. Thirty-seven persons (24.5%) had history of previous intensive FVIII treatment for surgical procedure. They received a mean perioperative cumulative FVIII of 498 iu/kg within first week after surgery. Seven cases (4.6%) developed an inhibitor post-operatively in our study. Surgical procedure for pseudotumor and the group of persons who experienced postoperative complications had the higher incidence of inhibitor development (9.5%, 13.3% respectively). Only previous history for intensive FVIII exposure was considered as a significant predictor for postoperative inhibitor development after multivariate logistic regression analysis (OR: 29.5, P = 0.002).
The incidence of inhibitor development in previously treated persons with HA undergoing major orthopaedic surgery was 4.6% and the history of previous intensive FVIII treatment for surgery was associated with higher risk of inhibitor development.
血友病 A (HA) 是一种 X 连锁隐性遗传性出血性疾病，由凝血因子 VIII 缺乏或缺乏引起。
本研究旨在确定先前接受过强化 FVIII 替代治疗的 HA 患者在接受重大骨科手术后，因抑制物发展而导致的发生率和治疗相关危险因素。
共回顾了 151 名接受过 221 次重大骨科手术的 HA 患者，这些患者均接受过强化 FVIII 治疗。收集抑制物检测结果。分析抑制剂发展的潜在临床危险因素。
111 人被诊断为严重 HA。37 人（24.5%）有过因手术接受过强化 FVIII 治疗的病史。他们在术后第一周内接受了平均每公斤 498 iu/kg 的围手术期累计 FVIII。在我们的研究中，有 7 例（4.6%）术后出现了抑制剂。假性肿瘤手术和术后出现并发症的患者组中，抑制剂发展的发生率更高（分别为 9.5%和 13.3%）。仅多变量逻辑回归分析显示，既往强化 FVIII 暴露史是术后抑制剂发展的显著预测因素（OR：29.5，P=0.002）。
先前接受过强化 FVIII 治疗的 HA 患者在接受重大骨科手术后发生抑制剂发展的发生率为 4.6%，且既往因手术接受强化 FVIII 治疗与抑制剂发展风险增加相关。
