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[肝移植作为重度甲型血友病潜在的治愈方法:病例报告及文献综述]

[Liver transplantation as potential curative method in severe hemophilia A: case report and literature review].

作者信息

Polák Pavel, Smejkal Petr, Romanová Gabriela, Zavřelová Jiřina, Hrdličková Radomíra, Blahutová Šárka, Husová Libuše, Zvarová Marta, Penka Miroslav

出版信息

Vnitr Lek. 2020 Spring;66(5):85-89.

Abstract

The authors present clinical case of orthotopic liver transplantation for cirhosis due to chronic viral hepatitis C in a subject with severe hemophilia A. Preoperatively performed pharmacokinetic study with recombinant F VIII confirmed satisfactory in vivo recovery of 2.1 %. A bolus application of 52 units F VIII/kg body weight with target F VIII activity over 100.0 % was administred shortly before the transplantation started. Totally, 30 000 units of recombinant F VIII, 3 thrombocyte concentrates, 2 erythrocyte concentrates, 5 units of virally inactivated plasma, 1 unit of fresh frozen plasma and 3 500 antithrombin units were used. There were no perioperative or postoperative bleeding complications, F VIII substitution was stopped on postoperative day 3. The patient was discharged on twentieth postoperative day.

摘要

作者介绍了一名患有严重甲型血友病的患者因慢性丙型病毒性肝炎肝硬化接受原位肝移植的临床病例。术前使用重组凝血因子VIII进行的药代动力学研究证实体内回收率为2.1%,令人满意。在移植开始前不久,静脉推注52单位/千克体重的凝血因子VIII,使凝血因子VIII活性超过100.0%。总共使用了30000单位的重组凝血因子VIII、3单位血小板浓缩物、2单位红细胞浓缩物、5单位病毒灭活血浆、1单位新鲜冰冻血浆和3500抗凝血酶单位。围手术期和术后均无出血并发症,术后第3天停止凝血因子VIII替代治疗。患者于术后第20天出院。

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