Department of Haemostasis & Thrombosis, Guy's & St Thomas' NHS Foundation Trust, London, UK.
Department of Trauma & Orthopaedics, Guy's & St Thomas' NHS Foundation Trust, London, UK.
Haemophilia. 2020 Jan;26(1):33-40. doi: 10.1111/hae.13870. Epub 2019 Nov 19.
The management of haemophilia-associated pseudotumours presents an ongoing challenge to the haematologist, surgeon and interventional radiologist alike. There is a range of therapeutic approaches including factor replacement, embolization, radiotherapy and a variety of surgical interventions. However, there remains little evidence regarding the most appropriate treatment. We aimed to evaluate the available options of management for the haemophilia-associated pseudotumour. A literature review was performed using relevant terminology and reviewed for treatment approaches and outcomes. The results demonstrated that most of the data is from single case reports with a small number of single- and multicentre case series. In total, 133 patients with 134 described pseudotumours were identified. Adequate haemostatic control with factor replacement was a key component to successful treatment. Surgical excision was the most commonly reported surgical intervention with various composites used for filling of the surgical cavity. The use of radiotherapy has been described particularly in the paediatric population and sites of difficult surgical access. Embolization can be considered as a method of presurgical optimization. Patients with both factor inhibitors and pseudotumours have poorer postoperative outcomes. This review demonstrates that although a lack of large-centre, randomized studies, timely surgical intervention with adequate haemostatic support and the consideration adjuvant therapies in selected cases can achieve acceptable outcomes in this cohort of patients.
血友病相关性假瘤的管理对血液科医生、外科医生和介入放射科医生来说都是一个持续存在的挑战。有一系列的治疗方法，包括因子替代、栓塞、放疗和各种手术干预。然而，关于最适当的治疗方法，仍然几乎没有证据。我们旨在评估血友病相关性假瘤的现有治疗选择。使用相关术语进行了文献回顾，并对治疗方法和结果进行了审查。结果表明，大多数数据来自于单个病例报告，少数为单中心和多中心病例系列。总共确定了 133 名患者的 134 个描述性假瘤。充分的因子替代止血控制是成功治疗的关键组成部分。手术切除是最常报道的手术干预方法，各种复合材料用于填充手术腔。放射治疗的应用已在儿科人群和手术难以进入的部位中得到描述。栓塞可以被认为是术前优化的一种方法。有因子抑制剂和假瘤的患者术后结局较差。这篇综述表明，尽管缺乏大型中心、随机研究，但及时的手术干预，充分的止血支持，以及在选定病例中考虑辅助治疗，可以为这组患者获得可接受的结果。
