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患有抑制剂的患者中的血友病假肿瘤

Haemophilia pseudotumours in patients with inhibitors.

作者信息

Caviglia H, Candela M, Landro M E, Douglas Price A L, Neme D, Galatro G A

机构信息

Department of Orthopaedic Surgery and Traumatology, Dr. Juan A. Fernández General Hospital, Buenos Aires, Argentina.

Haemophilia Foundation, Buenos Aires, Argentina.

出版信息

Haemophilia. 2015 Sep;21(5):681-5. doi: 10.1111/hae.12632. Epub 2015 Feb 16.

DOI:10.1111/hae.12632
PMID:25688657
Abstract

Development of inhibitors against factor VIII (FVIII) or FIX is the most serious complication of replacement therapy in patients with haemophilia. Haemophilic pseudotumours in a patient with inhibitors can lead to devastating consequences. The aim of this study is to show our experience in the treatment of 10 pseudotumours in 7 patients with inhibitors who were treated by the same multidisciplinary team in the period between January 2000 and March 2013. Seven severe haemophilia A patients were treated at the Haemophilia Foundation in Buenos Aires, Argentina, for 10 pseudotumours. Eight were bone pseudotumours and two soft tissue. All patients underwent imaging studies at baseline to assess the size and content of the lesion. The patients received Buenos Aires protocol as conservative treatment of their pseudotumours for 6 weeks, after which they were evaluated. Only one patient responded to conservative treatment. Surgery was performed on the others six patients, since their pseudotumours did not shrink to less than half their original size. Any bleeding in the musculoskeletal system must be treated promptly in order to prevent pseudotumours. When pseudotumours do appear in inhibitor patients, they can be surgically removed when patients received proper haemostatic coverage, improving the quality of life of these patients.

摘要

开发针对凝血因子 VIII(FVIII)或 FIX 的抑制剂是血友病患者替代疗法最严重的并发症。患有抑制剂的患者出现血友病性假肿瘤会导致灾难性后果。本研究的目的是展示我们在 2000 年 1 月至 2013 年 3 月期间,由同一多学科团队治疗的 7 例患有抑制剂的患者中 10 个假肿瘤的治疗经验。7 例重度甲型血友病患者在阿根廷布宜诺斯艾利斯的血友病基金会接受了 10 个假肿瘤的治疗。其中 8 个是骨假肿瘤,2 个是软组织假肿瘤。所有患者在基线时均接受影像学检查,以评估病变的大小和内容。患者接受布宜诺斯艾利斯方案作为其假肿瘤的保守治疗 6 周,之后进行评估。只有 1 例患者对保守治疗有反应。其他 6 例患者接受了手术治疗,因为他们的假肿瘤没有缩小到原来大小的一半以下。肌肉骨骼系统的任何出血都必须及时治疗,以预防假肿瘤。当抑制剂患者确实出现假肿瘤时,在患者获得适当的止血覆盖后,可以通过手术切除,从而改善这些患者的生活质量。

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