目的　讨论卡梅现象（KMP）的临床表现、诊断与治疗。方法　回顾分析2010年1月－2016年1月收治的13 例卡梅现象患儿的临床资料，并进行文献复习。 结果　13例患儿中男10例、女3例，发病年龄均<6个月。12例患儿有出血点、 瘀斑、黄疸和皮肤包块等皮肤表现， 1例表现为胸腔积液；病灶位置分布多样；血小板严重减少，纤维蛋白原减少，D-二聚 体升高；起病至确诊中位时间为60天。采用手术及糖皮质激素、心得安、干扰素、西罗莫司等药物治疗，10例缓解、 3例死 亡，其中6例给予西罗莫司治疗者疗效均满意。 结论　卡梅现象以血管肿瘤合并严重血小板减少及消耗性凝血异常为特 点，发病年龄小，起病至确诊时间较长；手术治疗疗效确切；西罗莫司或可成为卡梅现象的有效治疗手段。

　Objective　To improve understanding of the clinical manifestations, diagnosis and treatment of childhood Kasabach-Merritt phenomenon (KMP). Methods　The clinical data of 13 patients admitted for KMP to XXX from January 2010 to January 2016 was retrospectively analyzed, with a review of relevant literature. Results　The patients were 10 males and 3 females. The age of presentation varied from newborn to 5 months. 12 patients had cutaneous manifestations, like petechiae, ecchymosis, jaundice, skin masses, etc, 1 patient had pleural effusion. The location of lesions varied. The laboratory hallmark consists of profound thrombocytopenia and hypofibrinogenemia with elevated D-dimers. The median time from initial presentation to diagnosis was 60 days. After approaches like surgery, corticosteroids, propranolol, interferon, sirolimus, etc, 10 patients got remission while 3 patients died. 6 patients treated with sirolimushad complete response. Conclusions　KMP is characterized with vascular tumor, severe thrombocytopenia and consumptive coagulopathy. Clinically, KMP often presents with early-onset and delay in diagnosis. Surgery is an effective approach for KMP. Sirolimus appears to be a promising treatment for KMP.