De Pietri L, Masetti M, Montalti R, Begliomini B, Reggiani A, Barbieri E, Biagioni E, Marietta M, Romano A, Pasetto A, Gerunda G E
Division of Anesthesiology, University of Modena and Reggio Emilia, Modena, Italy.
Transplant Proc. 2008 Jul-Aug;40(6):2077-9. doi: 10.1016/j.transproceed.2008.05.054.
Hemophilia B is a congenital recessive disorder caused by deficiency of coagulation factor IX (FIX). Surgical procedures can be performed in patients with hemophilia using high-purity and/or recombinant FIX, which has been shown to be safe and effective in surgical hemostasis. Liver transplantation is the only potentially curative treatment available for these patients, providing a long-term phenotypic cure for hemophilia. End-stage liver disease together with hemophilia exposes patients to greater risks of bleeding complications during the perioperative period with consequent difficulties in managing coagulopathy. The limited experiences reported by different investigators and the various strategies for clotting factor replacement make it difficult to define a single approach with respect to the optimal dose and method of administering FIX to achieve perioperative hemostasis. The limits of plasma-based coagulation tests--prothrombin time, activated partial thromboplastin time--have made thromboelastography a valid alternative in this kind of surgery. It has been demonstrated to be a useful tool for real-time analysis of clot formation using a whole-blood assay format. Further, it accurately illustrates the clinical effects of procoagulant or anticoagulant interventions. In this article, we have described the usefulness of thromboelastography to monitor the ability of high-purity FIX supplementation to restore a normal coagulation state and to guide the perioperative administration of blood products in a successful orthotopic liver transplantation in a hemophilic patient with deficiencies of factors IX and X, presenting with hepatitis C virus-related cirrhosis and hepatocellular carcinoma.
血友病B是一种由凝血因子IX（FIX）缺乏引起的先天性隐性疾病。血友病患者可使用高纯度和/或重组FIX进行外科手术，已证明其在手术止血中安全有效。肝移植是这些患者唯一可能治愈的治疗方法，可为血友病提供长期的表型治愈。终末期肝病合并血友病会使患者在围手术期面临更大的出血并发症风险，从而导致凝血障碍管理困难。不同研究者报告的有限经验以及凝血因子替代的各种策略使得难以确定关于给予FIX以实现围手术期止血的最佳剂量和方法的单一方法。基于血浆的凝血试验（凝血酶原时间、活化部分凝血活酶时间）的局限性使得血栓弹力图在这类手术中成为一种有效的替代方法。已证明它是一种使用全血检测形式实时分析凝血形成的有用工具。此外，它能准确说明促凝或抗凝干预的临床效果。在本文中，我们描述了血栓弹力图在监测高纯度FIX补充恢复正常凝血状态的能力以及指导一名患有丙型肝炎病毒相关肝硬化和肝细胞癌且缺乏因子IX和X的血友病患者成功进行原位肝移植围手术期血液制品给药方面的有用性。
