Department of Surgery and Transplantation, Zurich University Hospital, Zurich, Switzerland.
Department of Medical Oncology and Hematology, Hemophilia Comprehensive Care Center, Zurich University Hospital, Zurich, Switzerland.
Swiss Med Wkly. 2023 Aug 15;153:40094. doi: 10.57187/smw.2023.40094.
Haemophilic pseudotumours are complications in patients with haemophilia A or B and result from locally repetitive bleeding, mainly in the musculoskeletal system. Abdominal haemophilic pseudotumours are exceptionally rare but may cause severe complications. This systematic review aimed to evaluate therapy strategies for symptomatic abdominal haemophilic pseudotumours.
We systematically searched three databases (Medline [PubMed], Web of Science and EMBASE) for publications published between 1995 and 2023. Two reviewers independently selected the studies, extracted data and performed a quality assessment using the JBI critical appraisal checklist.
From a total of 1199 articles, 39 articles describing 41 cases were included for final analysis. Conservative or interventional treatment was performed in 12 cases. In eight cases, a step-up to surgical therapy after interventional treatment was indicated. Primary surgical therapy was performed in 21 cases. Failure to cure was documented in 50% (n = 6) of patients treated in the first group, with a mortality rate of 16.6% (n = 2). Interventional therapy with a step-up to surgery showed no morbidity or mortality. Primary surgical resection documented favourable results in 66.6% (n = 14), with failure to cure in 9.5% (n = 2) and a mortality rate of 14.3% (n = 3).
Primary surgical resection can be a first-line therapy for symptomatic, abdominal haemophilic pseudotumours, whereas preoperative embolisation could be used as a bridging therapy before surgery, especially in emergency settings. Diagnostic biopsy and percutaneous drainage should be avoided to prevent complications.
血友病性假瘤是 A 型或 B 型血友病患者的并发症，由局部反复出血引起，主要发生在肌肉骨骼系统。腹部血友病性假瘤极为罕见，但可能引起严重并发症。本系统评价旨在评估有症状的腹部血友病性假瘤的治疗策略。
我们系统地检索了三个数据库（Medline[PubMed]、Web of Science 和 EMBASE），以获取 1995 年至 2023 年期间发表的文献。两名审查员独立选择研究、提取数据，并使用 JBI 批判性评估清单进行质量评估。
从总共 1199 篇文章中，纳入了 39 篇描述 41 例病例的文章进行最终分析。12 例采用保守或介入治疗。8 例介入治疗后需要逐步转为手术治疗。21 例采用原发性手术治疗。第一组治疗的 50%（n=6）患者记录未治愈，死亡率为 16.6%（n=2）。介入治疗加手术没有发生发病率或死亡率。原发性手术切除记录了 66.6%（n=14）的良好结果，未治愈的比例为 9.5%（n=2），死亡率为 14.3%（n=3）。
原发性手术切除可作为有症状的腹部血友病性假瘤的一线治疗方法，而术前栓塞可作为手术前的桥接治疗方法，特别是在紧急情况下。应避免诊断性活检和经皮引流，以防止并发症。
