Division of Pediatric and Adolescent Hematology/Oncology, Department of Pediatrics and Human Development, Michigan State University College of Human Medicine, East Lansing, MI 48824, USA.
Haemophilia. 2013 Jan;19(1):2-10. doi: 10.1111/j.1365-2516.2012.02922.x. Epub 2012 Aug 27.
Congenital haemophilia is a rare and complex condition for which dedicated specialized and comprehensive care has produced measurable improvements in clinical outcomes and advances in patient management. Among these advances is the ability to safely perform surgery in patients with inhibitor antibodies to factors VIII and IX, in whom all but the most necessary of surgeries were once avoided due to the risk for uncontrollable bleeding due to ineffectiveness of replacement therapy. Nevertheless, surgery continues to pose a major challenge in this relatively rare group of patients because of significantly higher costs than in patients without inhibitors, as well as a high risk for bleeding and other complications. Because of the concentration of expertise and experience, it is recommended that any surgery in patients with haemophilia and inhibitors be planned in conjunction with a haemophilia treatment centre (HTC) and performed in a hospital that incorporates a HTC. Coordinated, standard pre-, intra- and postoperative assessments and planning are intended to optimize surgical outcome and utilization of resources, including costly factor concentrates and other haemostatic agents, while minimizing the risk for bleeding and other adverse consequences both during and after surgery. This article will review the special considerations for patients with inhibitors as they prepare for and move through surgery and recovery, with an emphasis on the roles and responsibilities of individual members of the multidisciplinary team in facilitating this process.
先天性血友病是一种罕见且复杂的疾病，通过专门的、全面的护理，可以显著改善临床结果，并推动患者管理的进步。这些进步包括能够安全地为因子 VIII 和 IX 的抑制剂抗体患者进行手术，这些患者曾经因为替代治疗无效而导致不可控制的出血风险，除了最必要的手术外，几乎所有手术都被避免。然而，由于与无抑制剂患者相比，成本显著增加，以及出血和其他并发症的高风险，手术仍然是这群相对罕见患者的主要挑战。由于专业知识和经验的集中，建议任何患有血友病和抑制剂的患者的手术都应与血友病治疗中心（HTC）一起计划，并在一家整合了 HTC 的医院进行。协调、标准的术前、术中和术后评估和计划旨在优化手术结果和资源的利用，包括昂贵的因子浓缩物和其他止血剂，同时最大限度地降低手术期间和之后出血和其他不良后果的风险。本文将重点讨论抑制剂患者在准备和进行手术及康复过程中的特殊注意事项，强调多学科团队中各个成员在促进这一过程中的作用和责任。
