KHE或TA及其伴发KMP的发生机制研究进展  

Research advances on the pathogenesis of Kaposi form hemangioendothelioma or tufted angioma and its concomitant Kasabach-Merritt phenomenon

作　　者：张鲁鲁 赵雄[1] 岳晓洁[1] Zhang Lulu;Zhao Xiong;Yue Xiaojie(Department of Burns&Plastic Surgery,Affiliated Children's Hospital,Zhejiang University School of Medicine,National Clinical Research Center for Children's Health,Hangzhou 310052,China)

机构地区：[1]浙江大学医学院附属儿童医院烧伤整形科,国家儿童健康与疾病临床医学研究中心,杭州310052

出　　处：《中华小儿外科杂志》2021年第9期852-856,共5页Chinese Journal of Pediatric Surgery

摘　　要：Kasabach-Merritt现象(Kasabach-Merritt phenomenon,KMP)指由卡波西型血管内皮瘤(Kaposi form hemangioendothelioma,KHE)或丛状血管瘤(tufted hemangioma,TA)引起的,血小板减少和纤维蛋白原减少的消耗性凝血病,其发病年龄小,病情进展迅速,病死率高,严重危及患儿生命健康。该类疾病发病率低,目前具体发生机制不详,治疗效果欠佳,尚无统一的、高质量治疗指南以供参考。我们现对KHE或TA及其伴发KMP的发生机制做一综述,为临床治疗提供理论依据。Kasabach-Merritt phenomenon(KMP)is defined as Kaposiform hemangioendothelioma(KHE)or tufted angioma(TA)associated with fatal thrombocytopenia,together with hypofibrinogenemia and consumptive coagulopathy.With a young onset age and a high fatality rate,KMP progresses rapidly and the wellbeing of children are seriously endangered.The precipitating factors of KMP have remained elusive.The treatment outcomes are poor and there is no unified and high-quality treatment guidelines.This review summarized the pathogenesis of KHE or TA and its concomitant KMP to provide theoretical rationales for proper clinical treatments.

关 键 词：血小板 卡波西型血管内皮瘤 丛状血管瘤 

分 类 号：R732.2[医药卫生—肿瘤]