肝脏Kasabach-Merritt综合征1例报告及文献复习

Hepatic Kasabach-Merritt syndrome:A case report and literature review

目的探讨肝脏卡-梅综合征(Kasabach-Merritt syndrome,KMS)的临床特点及其诊疗方法。方法收集哈尔滨医科大学附属第一医院(1例,2009年10月)以及1984-2010年文献报道(50例)的肝脏KMS病人资料,对其发病情况、临床表现、实验室检查,治疗及预后进行回顾性分析。结果肝脏KMS的病理生理基础是与巨大血管肿瘤密切相关的血小板减少和弥散性血管内凝血(DIC),临床表现多样化,病理表现主要为海绵状血管瘤和血管内皮瘤。治疗方案包括纠正DIC和血小板减少,并根据情况选择外科治疗(手术切除、肝移植等)或药物治疗[糖皮质激素、干扰素(INF)等]去除血管肿瘤。结论肝脏血管肿瘤尤其是巨大血管肿瘤伴血小板减少症时,应警惕KMS的发生。一旦确诊,应在对症治疗的基础上,根据肿瘤的大小和部位,采用以手术或联合介入治疗为核心,以药物治疗及输血等多种方式相辅助的综合治疗。

Objective To investigate the clinical features, diagnosis and treatments of hepatic Kasabach-Merritt Syndrome （KMS）. Methods One case of hepatic KMS admitted in October 2009 in the First Affiliated Hospital of Harbin Medical University and the literature （50 cases） reported from 1984 to 2010 were collected. The incidence, clinical manifestations, laboratory tests, treatments and prognosis were analyzed retrospectively. Results The pathophysiological basis of KMS is thrombocytopenia which is closely related to the giant hemangioma and disseminated intravascular coagulation （DIC）. The clinical manifestations of the tumor are diversified and pathological manifestations are mainly cavernous hemangioma and hemangioendothelioma. Treatment procedure includes the correction of DIC and thrombocytopenia, surgical treatment （hepatectomy, liver transplantation, etc.）or pharmacotherapy （glucocorticoids, interferon, etc.）. Conclusion Hemangioma, especially giant hemangioma with thrombocytopenia, should be alert to hepatic KMS. Once the diagnosis is confirmed, the hepatic KMS should be given combined modality therapy according to the size and location of the tumor, the core of which is surgery and interventional therapy, and assisted by a variety of treatment modalities, such as pharmacotherapy, blood transfusions, etc.