婴幼儿Kasabach-Merritt综合征8例临床研究

Clinical investigation on 8 cases of infants with Kasabach-Merritt syndrome

目的探讨婴幼儿Kasabach-Merritt综合征(Kasabach-Merritt syndrome,KMS)的临床特征及治疗方法,为临床提供参考。方法 2013年1月至2016年12月,南方医科大学珠江医院儿科中心共收治8例婴幼儿KMS患儿。采用回顾性分析的方法,记录8例患儿的病史、临床表现、辅助检查结果、治疗方案及疗效等相关资料,并随访患儿预后。结果 8例患儿中,血管瘤分布分别为:颌下3例,下肢2例,内脏器官3例(肝脏、胰腺和肠系膜各1例)。6例患儿经药物综合治疗(糖皮质激素、α-2b干扰素、雷帕霉素和长春新碱等单药或联合治疗)后,瘤体缩小变软、颜色变淡,其中肠系膜和胰腺血管瘤患儿在药物治疗前分别因危及生命的瘤体内大出血和器官压迫接受缩瘤手术。血小板完全恢复正常的时间为4~30天,凝血功能完全恢复正常的时间为2~50天,瘤体完全消失的时间为1~2年。2例患儿因经济原因放弃治疗后死亡。存活的6例患儿随诊2年均治愈,身高、体重和器官功能均良好。结论婴幼儿KMS病情危重、进展迅速,糖皮质激素、长春新碱和雷帕霉素等联合治疗可快速控制出血症状,改善预后。对于发生危及生命的大出血、巨大血管瘤压迫重要器官的患儿,可考虑手术治疗以快速减少瘤体面积从而缓解出血。

Objective To investigate clinical features and therapeutic methods for infants with Kasabach-Merritt syndrome(KMS).Methods From January 2013 to December 2016,eight cases of infants with KMS were admitted to the Department of Pediatrics,Zhujiang Hospital of Southern Medical University.The medical history,clinical manifestations,results of laboratory tests,treatment methods and curative effect of eight cases were recorded by retrospective analysis method and the prognosis of the infants were followed up.Results Among the eight cases,the lesion location of angioma was three cases in submandibular,two cases in lower limbs,and three cases in internal organs(one case in liver,one case in pancreas,one case in mesentery).Angioma of six cases became shrunken and soft,and the color of the tumor became light after receiving drug therapy,which were glucocorticoid,α-2b interferon,rapamycin and vincristine single or combination therapy.Among them,mesenteric and pancreas angioma patients were treated with operation respectively because of life-threatening hemorrhage and giant hemangioma oppresses.The platelet was fully restored to normal in 4-30 days.The time of fully recovery of blood coagulation function was 2-50 days.The time of tumor completely disappeared was 1-2 years.Two cases due to economic reasons gave up treatment and died.Through two years follow-up,six cases were all cured.And the height,weight and organ function of the six cases were good.Conclusions Infants with KMS are in a critical condition and progress rapidly.Glucocorticoid,vincristine and rapamycin combined treatment can quickly control bleeding and improve prognosis.For the infants with critical bleeding and vital organs oppressed by huge angioma,surgery can quickly reduce tumor size so as to relieve bleeding.