Nilsson I M, Hedner U, Ahlberg A, Larsson S A, Bergentz S E
World J Surg. 1977 Jan;1(1):55-66. doi: 10.1007/BF01654733.
Seventy-seven hemophilic patients of type A or type B were subjected to a total of 108 major surgical procedures mainly in the field of general surgery, orthopedic surgery, or neurosurgery. The principles for the substitution therapy in the different types of procedures and different types of hemophilic diseases are described, as well as the indications for surgery and the surgical technique. The importance of prolonged substitution therapy postoperatively to avoid late hematoma, particularly in patients with severe hemophilia undergoing major surgery, is stressed. With this type of management there has been no increased intraoperative hemorrhage, and very few cases of late hematoma formation. By combining the substitution therapy with immunosuppression, it has been possible to operate also on patients with inhibitors against factor VIII or IX. The rate of complications, particularly the incidence of hepatitis, has been low with the type of substitution given in this series of patients. It is concluded that major surgery can be carried out even in severe hemophilia without significantly increased risk. The handling of the substitution therapy, and the surgical judgment and technique, offers however, special problems, necessitating centralization of elective cases.
77例甲型或乙型血友病患者共接受了108次主要外科手术，主要涉及普通外科、骨科或神经外科领域。文中描述了不同类型手术和不同类型血友病的替代疗法原则，以及手术指征和手术技术。强调了术后延长替代疗法以避免晚期血肿的重要性，特别是对于接受大手术的重型血友病患者。采用这种管理方式，术中出血没有增加，晚期血肿形成的病例也很少。通过将替代疗法与免疫抑制相结合，也能够对产生抗凝血因子VIII或IX抑制剂的患者进行手术。在这组患者中采用的替代疗法类型下，并发症发生率，特别是肝炎发生率较低。得出的结论是，即使是重型血友病患者也可以进行大手术，且风险不会显著增加。然而，替代疗法的处理以及手术判断和技术存在特殊问题，需要对择期病例进行集中管理。
