血友病性假肿瘤的临床特征和手术治疗:单中心20年的经验。

IF 2.4
Cheng-Fong Chen, Liang-Tsai Hsiao, Jan-Wei Chiu, Shang-Wen Tsai, Chao-Ming Chen, Po-Kuei Wu, Wei-Ming Chen
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引用次数: 0

摘要

背景:血友病假肿瘤(HPT)是血友病(PWH)患者中一种罕见但严重的并发症。鉴于其罕见性,病例系列很少,并且在文献中主要局限于病例报告。因此,本研究的目的是介绍一个研究所20年来HPT的手术结果。方法回顾性分析1999年至2020年在我院接受手术治疗的10例PWH患者中的11例HPT。确定了他们的临床特征、手术处理、结局和并发症。结果:10例男性PWH伴11例HPT, 20年间共接受了20次手术。手术时平均年龄为41.2岁,平均随访时间为9.7年。9例患者为A型血友病(5例重度、3例中度和1例轻度),1例患者为重度b型血友病。HPT定位于软组织2例(9.1%),骨内病变5例(45.5%),骨膜下病变4例(36.4%)。3例患者(27.2%)出现并发症,包括1例伤口感染、1例慢性骨髓炎和1例复发。9例HPT术后完全痊愈,1例复发,1例持续慢性骨髓炎,前者经进一步切除后病情稳定,后经截肢痊愈。结论:手术治疗HTP是可行和有效的,只要有适当的因素替代。鉴于各种各样,仔细的个案评估对于采取合适的手术方式和避免潜在的并发症是很重要的。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Clinical features and surgical treatment of hemophilic pseudotumors: A single-center experience over a 20-year period.

Background: Hemophilic pseudotumor (HPT) is an uncommon but severe complication in patients with hemophilia (PWH). Given the rarity, case series were scarce and largely confined to case report in the literature. Consequently, the aim of this study is to present surgical results of HPT over a 20-year period from a single institute.

Methods: We retrospectively reviewed 11 HPT in 10 PWH who underwent surgical treatment in our institute between 1999 and 2020. Their clinical features, surgical management, outcomes, and complications were identified.

Results: There are 10 men PWH with 11 HPT who had undergone a total of 20 surgical procedures over a 20-year period. The mean age at surgery was 41.2 years with an average follow-up of 9.7 years. Nine patients had hemophilia A (five severe, three moderate, and one mild) and one patient had severe hemophilia B. Localization of the HPT was confined to soft tissue in two patients (9.1%), to intraosseous lesion in five patients (45.5%) and to subperiosteal location in the remaining four patients (36.4%). Three patients (27.2%) had complications, including one wound infection, one chronic osteomyelitis, and one recurrence. Postoperative complete resolution was achieved in nine HPT except one recurrence and one persistent chronic osteomyelitis; the former was stable after further excision, and the latter was resolved by amputation.

Conclusion: Surgical therapy for HTP is feasible and effective when covered with adequate factor replacement. Given the variety, careful case-to-case evaluation is important to take the appropriate surgical modality and avoid potential complication.

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