Kasabach-Merritt综合征13例临床分析

Clinical Analysis on 13 Cases with Kasabach-Merritt Syndrome

目的了解Kasbach-Merritt综合征的临床特点,以减少误诊,并探讨其治疗方案。方法对本院2001年3月～2008年2月诊治的13例Kasabach-Merritt综合征患者的临床资料进行回顾性分析。结果13例患者中男4例,女9例,年龄均<6个月,均表现为不同部位较大的血管瘤,合并有血小板减少及出血症状,3例有弥漫性血管内凝血(DIC)。3例于大剂量糖皮质激素冲击治疗的基础上予支持对症治疗,效果较好。结论Kasabach-Merritt综合征临床少见,发病早,易误诊,DIC发生率高。根据患儿的皮损及实验室检查结果临床特点,采取不同的治疗方案,缓解病情,可为择期手术切除瘤体和局部注射硬化剂治疗赢得时机。

Objective To summarize the clinical characteristics of Kasbach-Merritt syndrome for reducing misdiagnosis, and to discuss its therapy. Methods The clinical data and therapeutic conditions of 13 patients diagnosed with Kasbach-Merritt syndrome from 2001.3 to 2008.2 were retrospectively analyzed. Results The 13 patients included 4 men and 9 women, with sex ratio is 1 ： 2.25. All the patients were infants less than 6 months or neonates featuring of hemangiomas varying in location and size combined with thrombocytopenia and hemorrhage, and 3 patients suffered from disseminated intra-vascular coagulation （ DIC ）. The therapy was based on high-dose corticosteroid shock, supplemented with supportive treatments. Conclusion Kasbach-Merritt syndrome is rare in clinic and easy to be misdiagnosed. It is likely complicated with DIC leading to death. According to patients＇ clinical characteristics, different therapeutic measures would be taken to relieve the state of illness and win time for regional injec- tion or surgical removal. Injection into mass body guided by typo-B ultrasonic is a new promising method to reduce drug dosage and side effects.