血管瘤伴血小板减少综合征11例

目的总结Kasabach-Merritt综合征的的临床特点,提高对本病的认识并探讨其治疗方案。方法对2002·4-2006·3年我院收治的11例Kasabach-Merritt综合征病人的临床特点、实验室检查、治疗情况进行回顾性分析。结果11例Kasabach-Merritt综合征中,男4例,女7例,年龄8天~5岁,临床特点为不同部位、大小的血管瘤伴不同程度血小板减少及出血症状,部分伴发骨骼畸形。治疗结果为1例手术治愈,8例药物治疗临床症状改善,2例药物治疗死亡。结论Kasabach-Merritt综合征临床少见,误诊率高,出血重,病死率相对较高。依据血小板减少程度不同,采取不同治疗方案,缓解病情,以提供适时的手术时机、对本症的预后尤为重要。

Kasabach-Merritt syndrome: eleven cases report

Objective To improve comprehension and treatment of Kasabach-Merritt syndrome (KMS) by summarizing the climcal characteristics of 11 cases retrospectively. Methods Eleven patients suffered with KMS and treated in Beijing Children Hospital between April 2002 and March 2006 were enrolled, and their clinical feature, laboratory test, therapy as well as response to treatment were analyzed. Results The eleven patients, four boys and seven girls, were eight days to five years old. Their manifestations included different sizes of hemangioma in different parts of body, accompany with thrombocytopenia and coagulopathy, some patients presented bone malformations. One patient was cured by rumor resection, eight patients improved by drug treatment, and two died of disseminated intravascular coagulation. Conclusion KMS is an infrequent but potentially fatal complication of rapidly growing hemangiomas especially in infants, It is more important to improve the condition of thrombocytopenia to get the chance for surgery, which would ameliorate the prognosis of the life-threatening symptom.