肝脏Kasabach-Merritt综合征的诊疗进展

摘要

Hepatic Kasabach-Merritt syndrome (KMS) is a highly complex and potentially fatal complication of severe hepatic hemangioma. Although hepatic KMS is rare,the death rate of victims is significant because it is often misdiagnosed upon presentation and thus receives improper treatment.Presently the literature lacks a systematic review of hepatic KMS.We have summarized its pathogenesis,diagnosis, systematic treatment, prognosis and outcomes in order to increase awareness of this deadly disease.%肝脏Kasabach-Merritt综合征是一种高潜在致死性复杂肝脏血管瘤的严重并发症.该综合征临床罕见,容易造成误诊、误治,甚至危及生命.目前该病缺乏系统性研究报道.为提高对该病的认识,本文就肝脏KasabachMerritt综合征的发病机制、诊断、综合治疗、预后和转归情况作一综述.