伴卡梅现象儿童血管瘤36例临床特点及治疗分析  

Analysis on clinical characteristics and treatment for Kasabach-Merritt phenomenon in 36 children with hemangioma

作　　者：黄世浩 袁晓军[1] HUANG Shihao;YUAN Xiaojun(Department of Pediatric Hematology/Oncology,Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine,Shanghai 200092,China)

机构地区：[1]上海交通大学医学院附属新华医院儿血液肿瘤科,上海200092

出　　处：《临床儿科杂志》2024年第11期917-921,934,共6页Journal of Clinical Pediatrics

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。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.

关 键 词：卡梅现象 临床特点 治疗 儿童 

分 类 号：R732.2[医药卫生—肿瘤]