【摘要】 目的 分析具有卡梅现象的儿童血管瘤患者的临床特点，总结治疗经验。方法 回顾性分析2006年4月1日至2021年12月31日就诊的伴有卡梅现象的儿童血管瘤患者的临床资料。结果 共36例血管瘤儿童伴有卡梅现象，占同期血管瘤就诊患儿的0.6%。5例(13.9%)患儿在发病早期出现典型卡梅现象，26例(72.2%)肿块位置浅表，瘤体小，4例(11.1%)肿块位于组织深部，瘤体相对较大，1例(2.8%)以便血为首发症状，肿块未被发现。中位随访时间为86个月。所有患儿确诊时中位血小板计数为24.5(11.8～43.5)×10~9/L，治疗后恢复至168.0(101.8～314.5)×10~9/L。24例接受综合治疗的患儿均获得完全缓解；12例接受单一治疗的患儿中，5例获得完全缓解，3例部分缓解，4例治疗无效。所有患儿的总体完全缓解率为80.6%，部分缓解率为8.3%，无效率为11.1%；7年总体生存率为88.9%，病死率为11.1%。结论 症状隐匿是卡梅现象发病的早期特点。瘤体进行性增大或伴有其他出凝血异常症状的血管瘤患者，需尽早完善相关实验室检查。更多还原

【Abstract】 Objective To analyze the clinical characteristics of pediatric hemangioma patients with Kasabach-Merritt phenomenon (KMP),and sum up its treatment experience.Methods The clinical data of children with KMP diagnosed from April 1,2006 to December 31,2021 were retrospectively analyzed.Results A total of 36 children with hemangioma were accompanied by KMP,accounting for 0.6% of the children with hemangioma.Five patients (13.9%) showed typical clinical manifestations of KMP in the early stage of the disease;26 patients (72.2%) had superficial tumors with small lesions;4 patients (11.1%) had deep-seated tumors with relatively larger lesions;1 patient (2.8%) presented with hematochezia as the initial symptom,and the tumor was not detected.Median follow-up time was 86 months.All patients manifested thrombocytopenia at the time of diagnosis and the median platelet counts were 24.5(11.8-43.5)×10~9/L,which increased to 168.0(101.8-314.5)×10~9/L after treatment.Twenty-four patients (66.7%) received combined therapy,all of them got complete remission (CR).Twelve patients (33.3%) received monotherapy,of them,five patients got CR,three got partial remission (PR),four showed no response.The overall CR rate was 80.6%,PR rate was 8.3%,and NR rate was 11.1%.The 7-year overall survival rate was 88.9% and the fatality rate was 11.1%.Conclusions The masking of symptom was the characteristics of KMP.Appropriate laboratory testing should be performed as soon as possible for patients with dysfunction of blood coagulation or rapidly progressive hemangiomas.更多还原