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α干扰素治疗难治性卡波西样血管内皮瘤:单中心经验

Interferon-alpha therapy for refractory kaposiform hemangioendothelioma: a single-center experience.

作者信息

Wu Hai Wei, Wang Xuan, Zhang Ling, Zhao Hai Guang, Wang Yan An, Su Li Xin, Fan Xin Dong, Zheng Jia Wei

机构信息

Department of Oral and Maxillofacial Surgery, Shanghai Ninth People's Hospital, College of Stomatology, Shanghai Jiao Tong University School of Medicine, Shanghai 200011, China.

Department of General Dentistry, Shanghai Ninth People's Hospital, College of Stomatology, Shanghai Jiao Tong University School of Medicine, Shanghai 200011, China.

出版信息

Sci Rep. 2016 Oct 31;6:36261. doi: 10.1038/srep36261.

Abstract

Kaposiform hemangioendothelioma (KHE) is a relatively rare vascular tumor with an aggressive and infiltrating nature. Previous studies have revealed an exclusive relationship between KHE and Kasabach-Merritt Phenomenon (KMP), which is associated with high morbidity and mortality. No universally accepted treatment modality exists for refractory KHE with or without KMP. The aim of this study was to evaluate the safety and efficacy of interferon-alpha (IFN-α) therapy for treatment of refractory KHE. Twelve consecutive patients with KHE were treated with subcutaneous injections of IFN-α after other treatments had failed. Eleven patients exhibited a reduction in tumor size of more than 50%, and the platelet count for all five patients with KMP returned to normal level after IFN-α therapy. The duration of IFN-α treatment ranged from 3 months to 9 months (mean: 6.3 months). The response time for IFN-α treatment ranged from 10 days to 5 weeks (mean: 3.6 weeks). Additionally, no severe complications, such as neurological damage or spastic diplegia, were observed in these patients. In conclusion, our study suggested that IFN-α therapy is effective and safe for refractory KHE, and IFN-α may be used as an alternative after other treatments have failed.

摘要

卡波西样血管内皮瘤(KHE)是一种相对罕见的具有侵袭性和浸润性的血管肿瘤。先前的研究揭示了KHE与卡萨巴赫-梅里特现象(KMP)之间的独特关系,KMP与高发病率和死亡率相关。对于伴有或不伴有KMP的难治性KHE,目前尚无普遍接受的治疗方式。本研究的目的是评估干扰素-α(IFN-α)治疗难治性KHE的安全性和有效性。在其他治疗失败后,连续12例KHE患者接受了皮下注射IFN-α治疗。11例患者肿瘤大小缩小超过50%,所有5例伴有KMP的患者在接受IFN-α治疗后血小板计数恢复正常水平。IFN-α治疗持续时间为3个月至9个月(平均:6.3个月)。IFN-α治疗的反应时间为10天至5周(平均:3.6周)。此外,在这些患者中未观察到严重并发症,如神经损伤或痉挛性双瘫。总之,我们的研究表明,IFN-α治疗难治性KHE有效且安全,在其他治疗失败后,IFN-α可作为一种替代治疗方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b08c/5087085/0fc5ab22fce6/srep36261-f1.jpg

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