糖皮质激素联合聚桂醇泡沫硬化剂治疗卡-梅综合征（现象）16例临床分析

中国口腔颌面外科杂志 ›› 2016, Vol. 14 ›› Issue (6): 557-561.

糖皮质激素联合聚桂醇泡沫硬化剂治疗卡-梅综合征（现象）16例临床分析

王亚飞, 王忠强

北京长峰医院婴幼儿血管瘤科,北京 100039

出版日期:2016-11-20 发布日期:2016-12-05
通讯作者: 王忠强,E-mail:doctorwzq@163.com
作者简介:王亚飞（1973-）,男,学士,主治医师,E-mail:wyf_030206@163.com

Glucocorticoid combined with lauromacrogol foam sclerotherapy for treating Kasabach-Merritt syndrome (phenomenon): a retrospective analysis of 16 cases

WANG Ya-fei, WANG Zhong-qiang

Department of Infantile Hemangiomas, Beijing Changfeng Hospital. Beijing 100039, China

Online:2016-11-20 Published:2016-12-05

摘要/Abstract

摘要： 目的评价糖皮质激素联合聚桂醇泡沫硬化剂治疗卡-梅综合征（现象）的临床疗效。方法 2013年1月—2014年12月间收治16例确诊的卡-梅综合征患儿,其中男7例,女9例;年龄最小5 d,最大1岁8个月,中位年龄3个月。瘤体位于头颈部8例,四肢6例,躯干部2例。16例患儿均首先采用静滴地塞米松注射液,0.5~0.75 mg/(kg·d),连用7 d,后改为口服泼尼松3~5 mg/(kg.d),隔天1次,晨起顿服,1个月后开始减量至停药;瘤体局部采用聚桂醇泡沫硬化剂注射治疗,每周1次,平均3~5次。治疗中密切观察患儿瘤体大小、硬度、色泽及温度变化,定期检测血小板计数。结果随访6个月~2 a,痊愈10例,显效3例,无效3例,总有效率达81.3%。结论糖皮质激素联合聚桂醇泡沫治疗卡-梅综合征疗效可靠,可明显降低激素的用量及服用时间,减少激素的副作用,易于推广应用。

关键词: 糖皮质激素, 聚桂醇泡沫, 卡-梅综合征, 卡-梅现象

Abstract: PURPOSE: To investigate the clinical efficiency of glucocorticoid combined with lauromacrogol foam sclerotherapy for treating Kasabach-Merritt syndrome (phenomenon, KMS, KMP). METHODS: Sixteen patients (7 males and 9 females) with KMS (KMP) admitted in our hospital from January 2013 to December 2014 were included in this study. The patients aged from 5 days to 20 months (median: 3 months). Eight patients showed lesion in head and neck, 6 in extremities and 2 in trunk. All patients received intravenous injection of dexamethasone (0.5-0.75 mg/kg per day) for 7 days, followed by oral prednisone (3-5 mg/kg per day) every 2 days. One month later, the dose was gradually decreased until termination. Local lesion was treated by lauromacrogol foam sclerotherapy once per week with a schedule of 3-5 times in total. The size, texture, color and temperature of the lesion were monitored, and blood platelet count was performed regularly. RESULTS: The follow-up period was 6-24 months. Ten patients showed complete response after treatment, 3 showed obvious response and 3 showed disease progression. The total effective rate was 81.3%. CONCLUSIONS: Glucocorticoid combined with lauromacrogol foam sclerotherapy is effective for treating KMS (KMP), which can remarkably decrease the dose and duration of corticosteroids therapy, minimized the side effects of corticosteroids. Such therapy deserves further application in clinical practice.

Key words: Glucocorticoid, Lauromacrogol foam sclerotherapy, Kasabach-Merritt syndrome, Kasabach-Merritt 557/img_1.jpg674.02.0phenomenon

中图分类号:

R732.2

王亚飞, 王忠强. 糖皮质激素联合聚桂醇泡沫硬化剂治疗卡-梅综合征（现象）16例临床分析[J]. 中国口腔颌面外科杂志, 2016, 14(6): 557-561.

WANG Ya-fei, WANG Zhong-qiang. Glucocorticoid combined with lauromacrogol foam sclerotherapy for treating Kasabach-Merritt syndrome (phenomenon): a retrospective analysis of 16 cases[J]. China J Oral Maxillofac Surg, 2016, 14(6): 557-561.

参考文献

[1] Hall GW. Kasabach-Merritt syndrome: pathogenesis and management [J]. Br J Haematol, 2001, 112(4): 851-862.
[2] Enjolras O, Wassef M, Mazoyer E, et al. Infants with Kasabach-Merritt syndrome do not have "true" hemangiomas [J]. J Pediatr, 1997, 130(4): 631-640.
[3] 周兆平, 李伟. 对“卡-梅现象”的认识 [J]. 组织工程与重建外科杂志, 2009, 5(3): 177-180.
[4] Thomson K, Pinnock R, Teague L, et al. Vincristine for the treatment of Kasabach-Merritt syndrome: recent New Zealand case experience [J]. N Z Med J, 2007, 120(1249): U2418.
[5] 李克雷, 秦中平, 刘学键, 等. 血管瘤和血管畸形伴发血小板减少综合征13 例分析 [J]. 上海口腔医学, 2005, 14(2): 108-111.
[6] 张志愿, 赵怡芳, 郑家伟, 等. 头颈部血管瘤与脉管畸形 [M]. 上海: 世界图书出版公司, 2007: 166-167.
[7] 袁斯明, 沈为民, 王修坤, 等. 11例中国儿童 Kasabach-Merritt现象的临床研究[J]. 组织工程与重建外科杂志, 2010, 6(3): 148-152.
[8] Hartman KR, Moncur JT, Minniti CP, et al. Mediastinal Kaposiform hemangioendothelioma and Kasabach-Merritt phenomenon in an infant: treatment with interferon [J]. J Pediatr Hematol Oncol, 2009, 31(9): 690-692.
[9] López V, Martí N, Pereda C, et al. Successful management of Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon using vincristine and ticlopidine [J]. Pediatr Dermatol, 2009, 26(3): 365-366.
[10] 刘小平, 郭伟, 贾鑫. 聚桂醇泡沫硬化剂治疗下肢静脉曲张的临床观察 [J]. 中国药物应用与监测, 2010, 7(2): 73-75.
[11] Breu FX, Guggenbichler S. European consensus meeting on foam sclerotherapy, April, 4-6, 2003, Tegernsee, Germany [J]. Dermatol Surg, 2004, 30(5): 709-717.
[12] Ikponmwosa A, Abbott C, Graham A, et al. The impact of different concentrations of sodium tetradecyl sulphate and initial balloon denudation on endothelial cell loss and tunica media injury in a model of foam sclerotherapy [J].Eur J Vasc Endovasc Surg, 2010, 39(3): 366-371.
[13] Jia X, Mowatt G, Burr JM, et al. Systematic review of foam sclerotherapy for varicose veins [J]. Br J Surg, 2007, 94(8): 925-936.
[14] Breu FX, Guggenbichler S, Wollmann JC. 2nd European consensus meeting on foam sclerotherapy 2006, Tegernsee, Germany [J].Vasa, 2008, 37(Suppl 71): 1-29.