Kasabach-Merritt现象诊断与治疗中国专家共识

doi:10.19438/j.cjoms.2019.02.001

中国口腔颌面外科杂志 ›› 2019, Vol. 17 ›› Issue (2): 97-105.doi: 10.19438/j.cjoms.2019.02.001

Kasabach-Merritt现象诊断与治疗中国专家共识

李克雷^1,*, 姚伟^2,*, 秦中平^1#, 李凯^2#, 郑家伟^3#, 范新东⁴, 马琳⁵, 周德凯⁶, 刘学键⁷, 尉莉⁵, 李丽⁵, 邰茂众¹, 王金湖⁸, 吉毅⁹, 周玲¹⁰, 黄海金¹¹, 高晓芸¹², 黄志见¹³, 顾松¹⁴, 杨合英¹⁵

1.山东省临沂市肿瘤医院省A级血管瘤特色专科,山东临沂 276001;
2.复旦大学附属儿科医院外科,上海 201102;
3.上海交通大学医学院附属第九人民医院·口腔医学院口腔颌面-头颈肿瘤科,上海 200011;
4.上海交通大学医学院附属第九人民医院介入科,上海 200011;
5.首都医科大学附属北京儿童医院皮肤科,北京 100045;
6.重庆医科大学附属儿童医院胃肠新生儿外科血管瘤专科,重庆 400014;
7.山东省临沂市经济技术开发区人民医院肿瘤科,山东临沂 276023;
8.浙江大学医学院附属儿童医院小儿外科,浙江杭州 310003;
9.四川大学华西医院小儿外科, 四川成都 610041;
10.新疆维吾尔自治区人民医院小儿外科,新疆乌鲁木齐 830001;
11.赣南医学院第一附属医院小儿外科,江西赣州 341000;
12.福建省立医院小儿外科,福建福州 350001;
13.苏州大学附属儿童医院小儿外科,江苏苏州 215025;
14.交通大学医学院上海市儿童医学中心小儿外科,上海 200127;
15.郑州大学第一附属医院小儿外科,河南郑州 450052

收稿日期:2019-01-08 修回日期:2019-02-10 出版日期:2019-03-20 发布日期:2019-04-12
通讯作者: 秦中平,E-mail: qinzhongping1962@163.com;李凯,E-mail: likai2727@163.com;郑家伟,E-mail: davidzhengjw@hotmail.com。^#共同通信作者
作者简介:李克雷（1971-）,男,硕士,主任医师,E-mail: likelei@sina.com;姚伟（1980-）,男,在读博士研究生,副主任医师,E-mail:2812094044@qq.com。^*并列第一作者

Chinese expert consensus on diagnosis and treatment of Kasabach-Merritt phenomenon

LI Ke-lei¹, YAO Wei², QIN Zhong-ping¹, LI Kai², ZHENG Jia-wei³, FAN Xin-dong⁴, MA Lin⁵, ZHOU De-kai⁶, LIU Xue-jian⁷, WEI Li⁵, LI Li⁵, TAI Mao-zhong¹, WANG Jin-hu⁸, JI Yi⁹, ZHOU Ling¹⁰, HUANG Hai-jin¹¹, GAO Xiao-yun¹², HUANG Zhi-jian¹³, GU Song¹⁴, YANG He-ying¹⁵

1. Special Department of Hemangioma, Tumor Hospital of Linyi City. Linyi 276001, Shandong Province;
2. Department of Pediatric Surgery, Children's Hospital of Fudan University. Shanghai 201102;
3.Department of Oral and Maxillofacial Surgery, Shanghai Ninth People's Hospital, College of Stomatology, Shanghai Jiao Tong University School of Medicine. Shanghai 200011;
4. Department of Intervention Therapy, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine. Shanghai 200011;
5.Department of Dermatology, Beijing Children's Hospital affiliated to Capital Medical University. Beijing 100045;
6. Department of Infantile Hemangioma, Gastrointestinal and Neonatal Surgery, Children's Hospital of Chongqing Medical University. Chongqing 400014;
7. Department of Oncology, People's Hospital of Linyi Economic Development Zone. Linyi 276023, Shandong Province;
8. Department of Pediatric Surgery, Children's Hospital, Zhejiang University School of Medicine. Hangzhou 310003, Zhejiang Province;
9. Department of Pediatric Surgery, West China Hospital, Sichuan University. Chendu 610041, Sichuan Province;
10. Department of Pediatric Surgery, People's Hospital of Xinjiang Uygur Autonomous Region. Urumchi 830001, Xinjiang Uygur Autonomous Region;
11. Department of Pediatric Surgery, First Affiliated Hospital of Gannan Medical University. Ganzhou 341000, Jiangxi Province;
12. Department of Pediatric Surgery, Fujian Provincial Hospital. Fuzhou 350001, Fujian Province;
13. Department of Pediatric Surgery, Children's Hospital of Soochow University. Suzhou 215025, Jiangsu Province;
14. Department of Pediatric Surgery, Shanghai Children's Medical Center. Shanghai 200127;
15. Department of Pediatric Surgery, The first Affiliated Hospital of Zhengzhou University. Zhengzhou 450052, Henan Province, China

Received:2019-01-08 Revised:2019-02-10 Online:2019-03-20 Published:2019-04-12

摘要/Abstract

摘要： Kasabach-Merritt现象（卡-梅现象）是主要由卡波西样血管内皮细胞瘤或丛状血管瘤所引起的严重血小板减少和消耗性凝血功能障碍为特征的一类疾病。该类疾病好发于婴幼儿,往往起病较急,病情进展迅速,若得不到及时诊治,可引起严重并发症,甚至危及生命。鉴于当前在卡-梅现象的诊断和治疗方面还存在一定分歧,为了提高国内对卡-梅现象的认识和诊治水平,规范临床治疗,我们组织国内相关专家讨论并制定了本专家共识。该共识对卡-梅现象的发病机制、临床表现、诊断方法及治疗流程等给出了规范化建议,可供临床实践参考。

关键词: Kasabach-Merritt现象, 卡波西样血管内皮细胞瘤, 丛状血管瘤, 专家共识

Abstract: Kasabach-Merritt phenomenon (KMP) is characterized by severe thrombocytopenia and consumptive coagulation dysfunction caused by Kaposiform hemangioendothelioma or tufted angioma. KMP is predominant in infants and little children and is often with rapid progress. If KMP is not diagnosed and treated in time, it can cause serious complications and even endanger life. In view of the current controversies in the diagnosis and treatment of KMP, and in order to improve the diagnosis level and standardize treatment of KMP in China, we invited relevant experts to discuss and formulate Chinese expert consensus. The consensus provides standardized suggestions on the pathogenesis, clinical manifestations, diagnostic methods and treatment process of KMP, which can provide reference for clinical practice.

Key words: Kasabach-Merritt phenomenon, Kaposiform hemangioendothelioma, Tufted angioma, Expert consensus

中图分类号:

R739.8

李克雷, 姚伟, 秦中平, 李凯, 郑家伟, 范新东, 马琳, 周德凯, 刘学键, 尉莉, 李丽, 邰茂众, 王金湖, 吉毅, 周玲, 黄海金, 高晓芸, 黄志见, 顾松, 杨合英. Kasabach-Merritt现象诊断与治疗中国专家共识[J]. 中国口腔颌面外科杂志, 2019, 17(2): 97-105.

LI Ke-lei, YAO Wei, QIN Zhong-ping, LI Kai, ZHENG Jia-wei, FAN Xin-dong, MA Lin, ZHOU De-kai, LIU Xue-jian, WEI Li, LI Li, TAI Mao-zhong, WANG Jin-hu, JI Yi, ZHOU Ling, HUANG Hai-jin, GAO Xiao-yun, HUANG Zhi-jian, GU Song, YANG He-ying. Chinese expert consensus on diagnosis and treatment of Kasabach-Merritt phenomenon[J]. China J Oral Maxillofac Surg, 2019, 17(2): 97-105.

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[5]	陈传俊, 陈伟良, 孙长伏, 何悦, 蒋灿华, 彭歆, 尚政军, 韩正学, 李劲松, 侯劲松, 林李嵩, 韩新光, 魏建华, 王志勇, 吴煜农, 郑家伟, 李龙江, 王慧明, 李祖兵, 郭传瑸, 杨驰, 石冰, 张志愿. 颏下岛状瓣修复口腔颌面部缺损专家共识[J]. 中国口腔颌面外科杂志, 2021, 19(5): 385-391.
[6]	何悦, 蒋灿华, 侯劲松, 王慧明, 彭歆, 刘冰, 李劲松, 郑磊, 徐中飞, 吴煜农, 胡镜宙, 秦兴军, 艾松涛, 周辉红, 李春洁, 张世周, 王军, 李晓光, 刘忠龙, 祝奉硕. 穿支皮瓣修复口腔颌面-头颈部缺损专家共识[J]. 中国口腔颌面外科杂志, 2020, 18(5): 385-389.
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Kasabach-Merritt现象诊断与治疗中国专家共识

Chinese expert consensus on diagnosis and treatment of Kasabach-Merritt phenomenon

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