新生儿Kasabach—Merritt综合征17例临床分析

Clinical analysis of 17 cases of neonatal Kasabach-Merritt syndrome

目的探讨新生儿Kasabach-Merdtt综合征（Kasabach．Merrittsyndrome，KMS）的临床特点与治疗方法。方法对2007年1月至2012年1月广州市妇女儿童医疗中心新生儿病区的17例KMS患儿l临床资料进行回顾性分析。结果17例中，男13例，女4例，年龄17h一28d，内脏血管瘤4例，皮肤表面血管瘤13例，入院时均有血小板减少及凝血功能异常。17例首先接受静脉激素治疗，激素敏感6例，无效11例，复发3例；随后对激素无效的11例及复发的3例行动脉硬化栓塞治疗，1例中途放弃治疗，8例有效，4例无效，1例复发；再对动脉栓塞治疗无效的4例及复发的1例加用长春新碱治疗，4例有效，1例并发弥散性血管内凝血死亡。本组激素治疗的敏感率为35．3％，但复发率为50％，动脉硬化栓塞术的有效率为61．5％。对激素及动脉硬化栓塞治疗无效的5例患儿使用长春新碱，有效率为80％。结论在新生儿KMS治疗中，糖皮质激素治疗抵抗常见。激素和动脉硬化栓塞联合治疗可作为新生儿KMS的一线治疗方案。上述治疗方法无效时，建议加用长春新碱。

Objective To summarize the clinical characteristics and therapeutic approaches of Ka- sabach-Merritt syndrome （KMS）. Methods The data from 17 KMS patients that had been definitely diagnosed from December,2007 to January,2012 in our tertiary center were collected retrospectively. Results We de- scribed 17 patients,13 of whom were male and 4 of whom were female,with an age range of 17 hours to 28 days. Of the 17 cases,4 cases had hemangioma of internal organs,13 on the surface of the body. All of them had thrombocytopenia and coagulation dysfunction. Seventeen cases were initially treated with corticosteroids. The re- sponses were varied： excellent and rapid improvement （ n = 6 ）, failure （ n = 11 ）, recurrence （ n = 3 ）. Then the cases of failure and recurrence accepted artery embolization： excellent and rapid improvement （ n = 8 ）, failure （ n = 4 ）, recurrence （ n = 1 ）, with 1 giving up. At last, the cases resistant to artery embolization therapy accepted vincristine therapy. Four cases had rapid improvement, and 1 died from disseminated intravascular coagulation. In this study, a response rate to corticosteroids was 35. 3 %, and the recurrent rate was 50%. The response rate to ar- tery embolization was 61.5%. Five patients unresponsive to hormone therapy and artery embolism were treated with vincristine,and the effective rate was 80%. Conclusion In the therapy of neonatal KMS,the resistant to corticosteroids is common. Combinative therapy of corticosteroids with artery embolization is recommended as the first-line therapy. Vincristine is suggested as a therapy when there is resistance to the other therapy.