Kasabach-Merritt现象诊断与治疗中国专家共识

Kasabach-Merritt现象（卡-梅现象）是主要由卡波西样血管内皮细胞瘤或丛状血管瘤所引起的严重血小板减少和消耗性凝血功能障碍为特征的一类疾病。该类疾病好发于婴幼儿,往往起病较急,病情进展迅速,若得不到及时诊治,可引起严重并发症,甚至危及生命。鉴于当前在卡-梅现象的诊断和治疗方面还存在一定分歧,为了提高国内对卡-梅现象的认识和诊治水平,规范临床治疗,我们组织国内相关专家讨论并制定了本专家共识。该共识对卡-梅现象的发病机制、临床表现、诊断方法及治疗流程等给出了规范化建议,可供临床实践参考。

Chinese expert consensus on diagnosis and treatment of Kasabach-Merritt phenomenon

Kasabach-Merritt phenomenon (KMP) is characterized by severe thrombocytopenia and consumptive coagulation dysfunction caused by Kaposiform hemangioendothelioma or tufted angioma. KMP is predominant in infants and little children and is often with rapid progress. If KMP is not diagnosed and treated in time, it can cause serious complications and even endanger life. In view of the current controversies in the diagnosis and treatment of KMP, and in order to improve the diagnosis level and standardize treatment of KMP in China, we invited relevant experts to discuss and formulate Chinese expert consensus. The consensus provides standardized suggestions on the pathogenesis, clinical manifestations, diagnostic methods and treatment process of KMP, which can provide reference for clinical practice.