摘要
目的 探讨Kasabach-Merritt（K-M）综合征的有效治疗方案。方法 收集2015—2017年首都医科大学附属北京儿童医院皮肤科收治的11例K-M综合征患儿的临床资料，分析长春新碱联合糖皮质激素治疗的疗效。结果 11例患儿就诊年龄1～212（87.91 ± 72.01） d，男4例、女7例。血管瘤多呈紫红色斑块，质地偏硬，其中5例瘤体周围伴有皮肤紫癜。血小板计数（4 ~ 32） × 109/L。给予口服泼尼松（2 ~ 5） mg·kg-1·d-1与静脉注射长春新碱每周0.05 mg/kg联合治疗。10例患儿应用糖皮质激素联合长春新碱治疗（1.8 ± 1.23）周后血小板达到正常，治疗（3.6 ± 1.26）周后纤维蛋白原恢复正常，治疗（3.9 ± 0.74）周后血管瘤开始软化或缩小。1例治疗5周后，血小板计数仍没有恢复正常，联合使用静脉栓塞后，血小板恢复正常，并可维持。结论 长春新碱联合糖皮质激素治疗可控制K-M综合征患儿瘤体发展，促进血小板恢复。
Objective To explore effective therapy for Kasabach-Merritt syndrome （KMS）. Methods Clinical data were collected from 11 infants with KMS in the Department of Dermatology, Beijing Children′s Hospital affiliated to Capital Medical University between 2015 and 2017, and the efficacy of vincristine combined with glucocorticoids for the treatment of KMS was evaluated. Results Of the 11 patients, 4 were male and 7 were female. Their average age at the first clinic visit was 87.91 ± 72.01 days （range, 1 - 212 days）. Most of the hemangiomas manifested as hard violaceous plaques, and skin purpura occurred around the hemangiomas in 5 patients. The initial blood platelet counts of the 11 patients ranged from 4 × 109/L to 32 × 109/L. All the patients were treated with oral prednisone at doses of （2 - 5） mg·kg-1·d-1 combined with intravenous injection of vincristine at a dose of 0.05 mg/kg every week. Among 10 patients treated with glucocorticoids and vincristine, the blood platelet counts became normal after （1.8 ± 1.23） weeks of treatment, the fibrinogen levels returned to normal after （3.6 ± 1.26） weeks of treatment, and the hemangiomas started to become softer and smaller after （3.9 ± 0.74） weeks. The blood platelet count still did not return to normal in 1 patient after 5 weeks of vincristine treatment. After the treatment with venous embolization, the blood platelet count gradually became normal and remained stable. Conclusion Vincristine combined with glucocorticoids can control the development of hemangioma in infants with KMS, and improve the recovery of blood platelet count.
作者
肖媛媛
徐子刚
徐哲
向欣
刘盈
马琳
杨舟
Xiao Yuanyuan;Xu Zigang;Xu Zhe;Xiang Xin;Liu Ying;Ma Lin;Yang Zhou(Department of Dermatology,Belting Children＇s Hospital,Capital Medical University,National Center for Children＇s Health,Beijing 100045,China)
出处
《中华皮肤科杂志》
CAS
CSCD
北大核心
2018年第8期601-603,共3页
Chinese Journal of Dermatology
基金
北京市科委“首都临床特色应用研究”专项课题（Z161100000516070）
北京市医院管理局临床医学发展专项经费（ZYLX201601）
