婴儿Kasabach-Merritt综合征16例临床回顾性分析

Clinical analysis of 16 cases with infantile Kasabach-Merritt syndrome

目的探讨婴儿Kasabach-Merritt综合征的临床特点、诊断及治疗方法。方法收集2008年1月—2014年12月诊治的16例Kasabach-Merritt综合征住院患儿,对其临床资料进行回顾性分析。结果 16例患儿男7例,女9例,平均年龄75.8 d,均表现为生后或生后不久出现迅速增大的血管瘤,并发血小板减少及出血倾向;均在采用糖皮质激素治疗的基础上支持对症治疗,9例联合局部外科治疗或瘤体内药物注射。12例治愈或好转,4例无效(其中3例死亡,1例失访)。结论 Kasabach-Merritt综合征发病早,病情危重,需要早期综合治疗,糖皮质激素仍为治疗的首选药物,多数患儿治疗效果较好。

Objective To investigate the clinical characteristics, diagnosis and treatment of infantile Kasabach-Merritt syndrome （KMS）. Methods Sixteen infants diagnosed as KMS were collected in Beijing Children’s Hospital of Capital Medical University from Jan. 2008 to Dec.2014, and the clinical data of them were studied retrospectively. Results There were 7 male and 9 female. The average age was 75.8 days. The common clinical manifestations were thrombocytopenic consumption coagulopathy associated with enlarging hemangioma, which found on birth or shortly after birth. The therapy of all the patients were based on corticosteroid, supplemented with supportive treatments, 9 cases took regional injection or vascular embolization. Conclusions KMS onset early and in a critical condition. Comprehensive treatment would be taken to alleviate the condition of the illness. Corticosteroids are still preferred effective therapeutic agents. Treatment effect is good for most cases.