Guys' and St Thomas' NHS Trust, London, UK.
Department of Translational Medicine, Lund University, Malmö, Sweden.
Haemophilia. 2021 May;27(3):e314-e330. doi: 10.1111/hae.14291. Epub 2021 Mar 10.
To assess available evidence on the use of rFVIIa in non-orthopaedic surgery including dental surgery in adult patients with congenital haemophilia with inhibitors (PWHI).
A systematic literature search was performed according to a prespecified search string; prespecified criteria were used to select applicable studies including PWHI ≥18 years of age who underwent any non-orthopaedic surgery using rFVIIa.
Thirty-three publications met the eligibility criteria, of which 26 publications - including 46 procedures in 44 patients - were selected for the qualitative analysis. Most publications were case reports or case series (21/26). Primary authors assessed rFVIIa as effective in maintaining haemostasis during and after most major surgeries (22/32). rFVIIa dose was mainly on label, with higher doses used in 4 cases, and a lower dose in 1 case. Duration of treatment was mostly 5-10 days (range: 3 days to 1 month post-operatively). Adverse events related to rFVIIa were rare.
Assessing non-orthopaedic surgery in this patient population is hampered by a paucity of published data; nevertheless, the current evidence indicates that rFVIIa is effective in achieving haemostasis in haemophilia patients with inhibitors undergoing elective non-orthopaedic or dental surgery. rFVIIa was generally well tolerated in these patients, with thrombotic events occurring rarely. These data, generated to help clinicians manage congenital haemophilia with inhibitors, highlight the need for more systematic reporting of rFVIIa and all other therapeutic agents in non-orthopaedic surgery and dental surgery in patients with congenital haemophilia and inhibitors.
评估在接受非骨科手术（包括成人牙科手术）的先天性血友病伴抑制物（PWHI）患者中使用 rFVIIa 的现有证据。
根据预先设定的检索词进行系统文献检索；使用预先设定的标准选择适用的研究，包括接受任何非骨科手术且使用 rFVIIa 的 PWHI≥18 岁的患者。
有 33 篇文献符合入选标准，其中 26 篇文献（44 名患者的 46 例手术）被纳入定性分析。大多数文献为病例报告或病例系列（21/26）。主要作者评估 rFVIIa 在大多数主要手术后的术中及术后维持止血方面是有效的（22/32）。rFVIIa 剂量主要为标签剂量，4 例使用了较高剂量，1 例使用了较低剂量。治疗持续时间主要为 5-10 天（术后 3 天至 1 个月）。与 rFVIIa 相关的不良事件罕见。
由于发表的数据较少，评估该患者人群的非骨科手术受到阻碍；然而，目前的证据表明，rFVIIa 可有效实现接受择期非骨科或牙科手术的血友病伴抑制物患者的止血。rFVIIa 在这些患者中通常具有良好的耐受性，很少发生血栓事件。这些数据有助于临床医生管理先天性血友病伴抑制物，强调需要更系统地报告 rFVIIa 及其他所有治疗药物在先天性血友病伴抑制物患者的非骨科手术和牙科手术中的应用。
