Department of Orthopaedics, Medical University of Vienna, Währinger Gürtel 18-20, 1090 Vienna, Austria.
Int Orthop. 2012 Oct;36(10):2157-62. doi: 10.1007/s00264-012-1593-7. Epub 2012 Jul 4.
Haemophilic pseudotumour was defined by Fernandez de Valderrama and Matthews as a progressive cystic swelling involving muscle, produced by recurrent haemorrhage into muscles adjacent to the bone. The pseudotumour mainly occurs in the long bones and the pelvis. The treatment of the haemophilic pseudotumour poses a challenge, and extensive clinical experience is essential to appropriately address this serious complication in patients with haemophilia. Consequently, the aim of this study is to present our own clinical experience and treatment results of the haemophilic pseudotumour.
We retrospectively reviewed the records of 87 patients with bleeding disorders treated between 1967 and 2011 for musculoskeletal complications of congenital bleeding disorders. We identified six patients with a haemophilic pseudotumour who were treated at our department.
The mean age at surgery was 45.9 (range, 40-61) years. The iliac bone was affected in three patients (one right, two left), the right tibia (distal diaphysis) in one, the right thigh in two and the right ulna (proximal part) in one patient. One patient had two pseudotumours. The perioperative course was easily controllable with adequate factor VIII substitution. At the latest follow-up after 8.4 (range, 4-24) years, normal healing with no recurrence was observed.
The haemophilic pseudotumour is a rare but severe complication of hereditary bleeding disorders. In the international literature the resection and postoperative course are described as challenging and difficult, requiring detailed preoperative planning. It is advisable to perform such operations in specialised centres with close co-operation between surgeons and haematologists.
Fernández de Valderrama 和 Matthews 将血友病假瘤定义为一种与骨骼相邻肌肉中反复出血导致的渐进性囊性肿胀。假瘤主要发生在长骨和骨盆。血友病假瘤的治疗颇具挑战，广泛的临床经验对于恰当地处理血友病患者的这种严重并发症至关重要。因此，本研究旨在介绍我们自己治疗血友病假瘤的临床经验和治疗结果。
我们回顾性分析了 1967 年至 2011 年间接受先天性出血性疾病肌肉骨骼并发症治疗的 87 例出血性疾病患者的记录，确定了在我们科室接受治疗的 6 例血友病假瘤患者。
手术时的平均年龄为 45.9 岁（范围 40-61 岁）。3 例患者（1 例右侧，2 例左侧）累及髂骨，1 例右侧胫骨（远段骨干），2 例右侧大腿，1 例右侧尺骨（近端）。1 例患者有 2 个假瘤。通过充分的 VIII 因子替代，围手术期过程易于控制。在最后一次随访时（8.4 年，范围 4-24 年），观察到正常愈合，无复发。
血友病假瘤是遗传性出血性疾病的一种罕见但严重的并发症。在国际文献中，切除和术后过程被描述为具有挑战性和困难，需要详细的术前计划。建议在有外科医生和血液科医生密切合作的专业中心进行此类手术。
