西罗莫司治疗卡波西形血管内皮瘤的研究进展<br />-《中国美容医学》

文章信息/Info

Title:: Research Progress of Sirolimus in Kaposiform Hemangioendothelioma Treatment

Keywords:: ; Key words: Kaposiform hemangioendothelioma; Kasabach-Merritt phenomenon; vascular anomalies; sirolimus

摘要:: [摘要]卡波西形血管内皮瘤（Kaposiform hemangioendothelioma,KHE）是一种罕见的婴幼儿血管内皮瘤，大部分患儿病情
进展迅速且无自发缓解趋势，其临床表现复杂多样，可伴随多种严重血液系统异常即Kasabach-Merritt现象（KMP），严重影响
患儿的生活质量甚至危及生命。目前的治疗方法主要有皮质醇激素、长春新碱化疗、干扰素、单抗、西罗莫司、普萘洛尔等，
西罗莫斯作为近年兴起的治疗药物，可从多方面缓解KHE的临床症状，改善患儿病情及生活质量，对于复杂性KHE的治疗有广泛
的临床应用前景。本文对比既往治疗方法与西罗莫司治疗KHE的特点，并探讨应用西罗莫司治疗KHE的机制研究与应用现况。

Abstract:: Abstract: Kaposiform hemangioendothelioma(KHE) was a rare vascular neoplasm that typically occurs during infancy and
childhood, adult cases were reported occasionally. Most cases were rapid and severe with complicated clinic signs, such as
Kasabach-Merritt phenomenon(KMP) which is fatal. In current, there are several treatments for KHE complicated by KMP,
such as corticosteroids, vincristine, interferon, propranolol and sirolimus. Sirolimus as a mTor blocker, is usually applied in
organ transplantation to reduce rejection reaction. To treat KHE, sirolimus displayed several advantages such as high efficiency
and light side effects. We reviewed related researches and case reports, aimed to discuss the mechanism and application of
sirolimus in KHE treatment.