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在手术期间及术后接受反复大剂量IX因子浓缩物治疗的B型血友病患者的高凝状态标志物。

Markers of hypercoagulability in patients with hemophilia B given repeated, large doses of factor IX concentrates during and after surgery.

作者信息

Santagostino E, Mannucci P M, Gringeri A, Tagariello G, Baudo F, Bauer K A, Rosenberg R D

机构信息

Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, IRCCS Maggiore Hospital, Milan, Italy.

出版信息

Thromb Haemost. 1994 Jun;71(6):737-40.

PMID:7974341
Abstract

Purer factor IX (FIX) concentrates have been produced for the treatment of hemophilia B in the attempt to reduce the risk of thrombotic complications associated with the use of prothrombin complex concentrates. To evaluate ex vivo whether or not FIX concentrates activate the coagulation system in conditions associated with a high risk for thrombosis, we measured markers of hypercoagulability in 10 patients with hemophilia B who underwent surgery, mainly orthopedic procedures, covered by multiple concentrate infusions (40-80 U/kg/day). Postinfusion plasma levels of prothrombin fragment 1 + 2 and factor X activation peptide did not differ significantly from the presurgical levels, neither before nor after each concentrate dose. Therefore, it appears that prolonged treatment of patients with hemophilia B undergoing high risk surgical procedures with high doses of FIX concentrate does not cause systemic activation of coagulation. This suggests that purified FIX concentrates are preferable to prothrombin complex concentrates for conditions associated with an increased risk of thrombosis.

摘要

为降低使用凝血酶原复合物浓缩物相关的血栓形成并发症风险,已生产出更纯的凝血因子IX(FIX)浓缩物用于治疗B型血友病。为在体外评估FIX浓缩物在血栓形成高风险情况下是否激活凝血系统,我们对10例接受手术(主要是骨科手术)的B型血友病患者进行了检测,这些患者接受了多次浓缩物输注(40 - 80 U/kg/天)。输注后血浆中凝血酶原片段1 + 2和因子X激活肽水平与术前水平相比,在每次浓缩物剂量前后均无显著差异。因此,对于接受高风险外科手术的B型血友病患者,长时间高剂量使用FIX浓缩物似乎不会引起全身凝血激活。这表明,在血栓形成风险增加的情况下,纯化的FIX浓缩物比凝血酶原复合物浓缩物更可取。

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