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目的:总结卡梅现象(KMP)患儿的临床特点及诊断与治疗情况。方法:回顾性分析2016年1月至2019年1月首都医科大学附属北京儿童医院血液肿瘤中心收治的8例KMP患儿的临床资料及实验室检查、诊疗过程、治疗及预后。结果:8例KMP患儿中，男6例，女2例；中位起病年龄4(0～17)个月，新生儿期起病2例；中位起病至诊断时间59(34～140) d；6例并骨破坏；6例既往有误诊误治病史，误诊为血小板减少性紫癜、伊文氏(Evans)综合征、骨关节发育异常；4例病理为卡波西样血管内皮瘤；8例患儿单用或联合应用激素、西罗莫司、长春新碱治疗，7例行介入治疗。预后：中位随访时间487(112～1 033) d，所有患儿均存活，血小板恢复正常中位时间24.5(7～60) d，纤维蛋白原恢复正常中位时间20(7～30) d，D-二聚体下降至正常中位时间105(40～240) d。结论:临床上对于并发血小板计数及凝血异常患儿需注意考虑KMP，临床医师需鉴别有无潜在内脏血管性病变因素，早期诊断及治疗对于改善患儿的临床预后有重要意义。“,”Objective:To analyze the clinical characteristics and diagnosis and treatment of children with Kasabach-Merritt phenomenon (KMP).Methods:A retrospective analysis was conducted on the clinical data and follow-up data of 8 patients diagnosed KMP in Beijing Children′s Hospital, Capital Medical University from January 2016 to January 2019.The clinical data included laboratory examination, diagnosis, treatment and prognosis.Results:Among the 8 children with KMP, 6 cases were male and 2 cases were female.The median onset age was 4 (0-17) months, 2 cases of neonatal onset.The median onset to the diagnosis time was 59 (34-140) days; 6 cases with bone destruction; 6 cases had misdiagnosis and mistreatment history, they were misdiagnosed as idiopathic thrombocytopenic purpura, Evans syndrome, abnormal bone and joint development; 4 cases were Kaposiform hemangioendothelioma; 8 cases were used alone or combined with the application of hormones, Sirolimus, and Vincristine, 7 patients underwent interventional therapy.All patients survived with a median follow-up period of 487 (112-1 033) days.Median time of platelet count returned to normal was 24.5 (7-60) days, and median time of fibrinogen returned to normal was 20 (7-30) days.Median time of D-dimer dropped to a normal was 105 (40-240) days.Conclusions:Children with concurrent platelet count and coagulation abnormalities should be considered with KMP.Doctors need to identify the potential visceral vascular lesions.Early diagnosis and treatment are important, which can improve the clinical prognosis of patients.