Management of elective procedures in low von Willebrand factor patients in the LoVIC study
Management of elective procedures in low von Willebrand factor patients in the LoVIC study
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DOI：
10.1111/jth.15220
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发表时间：
2021-01-24
影响因子：
10.4
通讯作者：
O'Donnell, James S.
中科院分区：
文献类型：
--
作者：
Doherty, Dearbhla;Lavin, Michelle;O'Donnell, James S.
背景：大多数轻度至中度血浆血管性血友病因子（VWF）水平降低的个体不会表现出出血增加。然而，一些血浆VWF水平为30-50 IU/dl的患者确实有明显的出血表型。由于缺乏证据，这些“低VWF”患者的管理仍然是一个共同的临床挑战，他们可能有明显的出血评分bbb10，大约是选择性手术的时间。目的探讨成人低VWF患者围手术期不同治疗方案的安全性和有效性。方法回顾性回顾了60例低VWF患者的160例侵入性手术的治疗和结果，这些患者纳入了先前描述的低血管性血友病因子爱尔兰队列研究。结果我们证明，1-去氨氨基-8- d -精氨酸抗利尿素在成人低VWF患者的小或大选择性手术中都能有效预防出血，即使是那些有明显出血史的患者。此外，氨甲环酸单独对低VWF患者进行非牙科小手术是有效的。重要的是，年龄相关的血浆VWF：抗原水平高于50 IU/dl并不一定与出血表型的完全纠正相关。低VWF患者术前未接受任何止血覆盖，手术相关出血并发症增加。结论成人低VWF患者的择期手术应与综合护理三级转诊中心联系管理，以便在所有小、大择期手术前实施个性化的治疗方案。
Background Most individuals with mild to moderate reductions in plasma von Willebrand factor (VWF) levels do not demonstrate increased bleeding. However, some patients with plasma VWF levels of 30-50 IU/dl do have a significant bleeding phenotype. Management of these "low VWF" patients, who may have significant bleeding scores >10, around times of elective procedures continues to pose a common clinical challenge because of a lack of evidence.Objective To investigate the safety and efficacy of different periprocedural management options for adult patients with low VWF.Methods Treatment and outcomes were retrospectively reviewed for 160 invasive procedures performed in 60 patients with well characterized low VWF enrolled in the previously described Low Von Willebrand factor Ireland Cohort study.Results We demonstrate that 1-desamino-8-D-arginine vasopressin is efficacious in preventing bleeding for both minor or major elective procedures in adult low VWF patients, even in those with significant bleeding histories. In addition, tranexamic acid alone is effective for low VWF patients undergoing nondental minor procedures. Importantly, age-related increases in plasma VWF:antigen levels above 50 IU/dl were not necessarily associated with complete correction of bleeding phenotype. Procedure-related bleeding complications were increased in low VWF patients who did not receive any hemostatic cover before their procedure.Conclusion Elective procedures in adult patients with low VWF should be managed in liaison with a comprehensive care tertiary referral center so that personalized treatment plans may be implemented before all minor or major elective procedures.