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使用mTOR抑制剂西罗莫司治疗淋巴管畸形:一项系统评价。

Treatment of Lymphatic Malformations with the mTOR Inhibitor Sirolimus: A Systematic Review.

作者信息

Wiegand Susanne, Wichmann Gunnar, Dietz Andreas

机构信息

Department of Otolaryngology, Head and Neck Surgery, University of Leipzig , Leipzig, Germany .

出版信息

Lymphat Res Biol. 2018 Aug;16(4):330-339. doi: 10.1089/lrb.2017.0062. Epub 2018 Jun 20.

Abstract

BACKGROUND

Extensive lymphatic malformations are low-flow vascular malformations that can cause devastating complications. Treatment of these malformations is challenging. This systematic review presents current use of sirolimus in patients with extensive lymphatic malformations.

METHODS

MEDLINE and Google scholar search was conducted for studies on sirolimus treatment of lymphatic malformations up to July 2017. Search items included "lymphatic malformation," "lymphangioma," "cystic hygroma," "vascular malformation," "low-flow malformation," "sirolimus," "rapamycin," and "mTOR inhibitor."

RESULTS

Twenty studies, including 71 patients receiving sirolimus, were included into this review. Forty-five patients had lymphatic malformations, eight patients venolymphatic malformations, and 19 patients capillary-lymphatico-venous malformations. Sirolimus led to a partial remission of disease in 60 patients, three patients had a progressive disease, and the outcome of eight patients was not reported. Dosing, target trough level, and duration of treatment differed between the studies. Common adverse effects were hyperlipidemia and neutropenia.

CONCLUSIONS

Available literature indicated that sirolimus therapy might be effective for lymphatic malformations. However, further randomized controlled studies are required to analyze the efficacy and long-term adverse events and to clarify the potential role for sirolimus in the management of lymphatic malformations.

摘要

背景

广泛淋巴管畸形是低流量血管畸形,可导致严重并发症。这些畸形的治疗具有挑战性。本系统评价介绍了西罗莫司在广泛淋巴管畸形患者中的当前应用情况。

方法

对截至2017年7月的西罗莫司治疗淋巴管畸形的研究进行了MEDLINE和谷歌学术搜索。搜索词包括“淋巴管畸形”、“淋巴管瘤”、“囊状水瘤”、“血管畸形”、“低流量畸形”、“西罗莫司”、“雷帕霉素”和“mTOR抑制剂”。

结果

本评价纳入了20项研究,包括71例接受西罗莫司治疗的患者。45例患者患有淋巴管畸形,8例患者患有静脉淋巴管畸形,19例患者患有毛细血管淋巴管静脉畸形。西罗莫司使60例患者病情部分缓解,3例患者病情进展,8例患者的结果未报告。各研究之间的给药剂量、目标谷浓度和治疗持续时间有所不同。常见不良反应为高脂血症和中性粒细胞减少。

结论

现有文献表明,西罗莫司治疗可能对淋巴管畸形有效。然而,需要进一步的随机对照研究来分析其疗效和长期不良事件,并阐明西罗莫司在淋巴管畸形管理中的潜在作用。

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