Kasabach-Merritt综合征38例回顾性分析

Retrospective analysis of 38 cases of Kasabach-Merritt Syndrome

目的了解Kasabach-Merritt综合征的临床特点,以减少误诊;并比较不同治疗方法对Kasbach-Merritt综合征的疗效,探讨最佳的治疗方案。方法对本院1990年3月-2010年12月诊治的38例Ka-sabach-Merritt综合征患儿的临床资料进行回顾性分析,将2005年12月以前收治的20例患儿设为对照组,予尿素联合激素治疗;2006年1月后收治的18例患儿设为治疗组,予长春新碱联合平阳霉素治疗。结果 38例患者中,男15例,女23例,年龄35天～9个月,平均3.6个月;临床均表现为不同部位的巨大血管瘤,合并严重的血小板减少及出血症状,3例有弥漫性血管内凝血(DIC)。对照组治愈17例,死亡3例,治疗组治愈17例,另1例继续治疗中,但瘤体明显缩小,血小板已恢复正常,且未再出现出血情况。结论 Kasabach-Merritt综合征临床少见,应根据患儿的病史、查体、实验室检查结果及影像学检查尽早确诊,降低误诊率,并采取不同的治疗方案尽早诊治,这对预后起关键作用。激素治疗是国际公认疗法,但是长春新碱联合平阳霉素治疗值得临床推广应用。

Objective To analyze clinical features of Kasabach-Merritt syndrome and compare the effectiveness of different treatment and to find the more effective therapeutic methods. Methods The clinical data of 38 patients with Ka- sabach-Merritt Syndrome between March 1990 and December 2010 were collected for retrospective analysis. The children were divided into 2 groups. Twenty cases before December 2005 treated with combination of urea and glucocorticosteroid were named as group one. Eighteen cases after January 2006 treated with combination of vin- cristine and pingyangmycin were named as group two. Results Thirty-eight cases including 15 males and 25 fe- males aged from 35 days to 9 months （mean 3.6 months）. All the cases showed large hemangioma of variable locations, thromytoipenia and hemorrhage, and 3 of them showed disseminated intravascular coagulation （DIC）. In group one, 17 cases recovered and 3 died from hemorrhage. In group two, 17 cases have recovered, 1 case is still on treatment and the hemangioma has regressed significantly, the platelet count has already normalized and there was no new hemorrhage in this case. Conclusions Kasabach-Merritt Syndrome is an unusual clinical condition, and the diagnosis should be established as early as possible. Usually the diagnosis must rely on history, physical examination, laboratory investigation and image study simultaneously. According to the condition of indi- vidual, we should choose the most proportionate treatment. Glucocorticosteroid therapy is a widely accepted treatment option in the world. In this study, combination of vincristine and pingyangrnycin showed impressive results for the treatment of KMS, and it should be advocated to spread more widely.