Management of orthopaedic surgery in rare bleeding disorders
Management of orthopaedic surgery in rare bleeding disorders
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DOI：
10.1111/hae.12387
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发表时间：
2014-09-01
期刊：
影响因子：
3.9
通讯作者：
Peyvandi, F.
中科院分区：
文献类型：
--
作者：
Siboni, S. M.;Biguzzi, E.;Peyvandi, F.
关于罕见出血性疾病（RBD）患者骨科手术管理的知识有限。1982年至2013年期间，对22例RBD患者进行了35例骨科手术（6例小手术和29例大手术）的回顾性数据收集和分析。除了4例轻度凝血因子缺乏症患者的7例手术未进行止血治疗外，这些手术均采用了不同的止血治疗方案。在28例进行止血治疗的手术中，9例（32%）使用补充疗法，使用浓度不足的因子，旨在达到与止血相容的围手术期血浆水平; 3例（11%）使用与新鲜冷冻血浆（FFP）相关的因子补充疗法; 4例（14%）使用重组活化因子VII; 4例（14%）仅使用病毒灭活血浆; 3例（11%）使用病毒灭活血浆联合去氨加压素; 1例（4%）仅使用FFP; 4例（14%）仅使用氨甲环酸。涉及5名患者的35例手术中有7例（20%）发生出血并发症。仅在1例无过度出血的病例中进行了静脉血栓栓塞预防，但2例未进行血栓预防的患者发生了血栓性浅静脉炎。大多数患者的止血控制令人满意。在一些以轻度因子缺乏（FVII、FXI）为特征的患者中，在某些情况下可以避免止血治疗。控制止血并结合适当的手术技术是RBD骨科手术成功结局的必要条件，需要专业血友病中心的参与。
Knowledge regarding the management of orthopaedic surgery in patients with rare bleeding disorders (RBDs) is limited. Retrospective data collection and analysis of 35 orthopaedic procedures (6 minor and 29 major) carried out in 22 patients with RBD between 1982 and 2013. These surgeries were performed using heterogeneous regimens of hemostatic therapy, except for seven procedures performed with no hemostatic treatment in four patients with mild factor deficiency. Of the 28 procedures carried out with hemostatic treatment, nine (32%) were performed using replacement therapy with dosages of concentrates of the deficient factor aimed to achieve perioperative plasma levels judged to be compatible with hemostasis; three (11%) using factor replacement therapy associated with fresh frozen plasma (FFP); four (14%) using recombinant activated factor VII; four (14%) using virus inactivated plasma alone; three (11%) using virus inactivated plasma associated with desmopressin; one (4%) using FFP alone; and four (14%) procedures using tranexamic acid alone. Bleeding complications occurred in 7 of 35 procedures (20%) involving five patients. Prophylaxis of venous thromboembolism was performed only in one case with no excessive bleeding, but two patients not on thromboprophylaxis developed superficial thrombophlebitis. A satisfactory control of hemostasis was achieved in most patients. In some of those characterized by mild factor deficiency (FVII, FXI) hemostatic treatment could be avoided in some instances. The control of hemostasis combined with an adequate surgical technique is needed for the successful outcome of orthopaedic surgery in RBDs that requires the involvement of specialized haemophilia centres.