血友病肌肉血肿的并发症。

Q2 Medicine
E Carlos Rodriguez-Merchan
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引用次数: 6

摘要

预防是必不可少的,以避免血友病患者肌肉血肿的并发症(室综合征,假性肿瘤和周围神经病变)。这是通过肌肉血肿的早期诊断和适当的长期血液学治疗来实现的,直到它们消失(通过图像研究证实)。超声引导下的经皮引流可以更好更快地缓解症状。急性筋膜室综合征(ACS)需要紧急手术治疗(减压筋膜切开术)。对于假肿瘤,活检将帮助我们确认诊断并排除真肿瘤(软骨肉瘤,脂肪肉瘤,滑膜肉瘤),有时类似血友病假肿瘤。手术切除血友病假瘤是最好的解决办法。作为替代,有刮除和填充松质骨和放射治疗(当手术禁忌时)。术前动脉栓塞(理想术前2周)有助于控制巨大盆腔假瘤术中出血。周围神经损伤,这是罕见的,几乎总是发生由于压迫附近的血肿。在大多数情况下,它们通常只能通过血液治疗来解决。如果这种治疗失败,就需要进行手术。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Complications of Muscle Hematomas in Hemophilia.

Prevention is essential for avoiding the complications of muscle hematomas (compartment syndrome, pseudotumors and peripheral nerve lesions) in hemophilic patients. This is achieved through early diagnosis of muscle hematomas and proper long-term hematological treatment until they have resolved (confirmed by image studies). Ultrasound-guided percutaneous drainage could be beneficial in terms of achieving better and faster symptom relief. Acute compartment syndrome (ACS) requires emergency surgical treatment (decompression fasciotomy). As for pseudotumors, the biopsy will help us confirm the diagnosis and rule out true tumors (chondrosarcoma, liposarcoma, synovial sarcoma) that sometimes mimic hemophilic pseudotumors. Surgical removal of hemophilic pseudotumors is the best solution. As alternatives, there are curettage and filling with cancellous bone and radiotherapy (when surgery is contraindicated). Preoperative arterial embolization (ideally 2 weeks before surgery) helps control intraoperative bleeding during surgery for giant pelvic pseudotumors. Peripheral nerve injuries, which are rare, almost always occur due to compression of hematomas in the vicinity. In most cases, they usually resolve with hematological treatment only. If such treatment fails, surgery would be indicated.

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来源期刊
Cardiovascular and Hematological Disorders - Drug Targets
Cardiovascular and Hematological Disorders - Drug Targets Medicine-Cardiology and Cardiovascular Medicine
CiteScore
1.90
自引率
0.00%
发文量
36
期刊介绍: Cardiovascular & Hematological Disorders - Drug Targets aims to cover all the latest and outstanding developments on the medicinal chemistry, pharmacology, molecular biology, genomics and biochemistry of contemporary molecular targets involved in cardiovascular and hematological disorders e.g. disease specific proteins, receptors, enzymes, genes. Each issue of the journal contains a series of timely in-depth reviews written by leaders in the field covering a range of current topics on drug targets involved in cardiovascular and hematological disorders. As the discovery, identification, characterization and validation of novel human drug targets for cardiovascular and hematological drug discovery continues to grow.
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