11例中国儿童Kasabach-Merritt现象的临床研究

目的分析11例中国儿童Kasabach-Merritt现象的临床数据。方法 2005年1月至2009年6月期间,11例具有KMP的血管肿瘤患者（男5例,女6例）,在南京市儿童医院整形外科和南京军区南京总医院整形外科接受治疗。收集以上病例的一般资料、血液检验、影像学检查和病理检查结果 ,进行回顾性分析。结果血液检验结果提示3例符合弥漫性血管内凝血（DIC）,其他为非典型DIC。CT检查提示团块状或弥漫性低密度影,增强扫描强化明显,可见粗大、扭曲的供血动脉进入肿块内。8例患者经手术切除或手术与长春新碱化疗结合等治疗,其中6例成活。这8例患者的病理检查结果均为卡波西样血管内皮瘤。死亡5例,死因为广泛出血和继发性多脏器功能衰竭。结论中国儿童的Kasabach-Merritt现象临床表现典型,原发血管肿瘤以卡波西样血管内皮瘤为主。手术结合化疗是治疗Kasabach-Merritt现象的有效方法 。

Clinical Study on 11 Cases of Kasabach-Merritt Phenomenon in Chinese Children

Objective To analyze the clinical data on 11 cases of kasabach-Merritt phenomenon （KMP） in Chinese children. Methods Between January 2005 and June 2009,11 patients with vascular tumor and KMP, 5 males and 6 females, were treated in Department of Plastic Surgery, Nanjing Children′s Hospital and Department of Plastic Surgery, Jinling Hospital. Their general information, and haematological, pathological, and imaging results were collected for retrospective analysis. Results Haematological test showed that, three patients had typical disseminated intravascular coagulation （DIC）, while the others had atypical DIC. CT scanning showed masses or diffused low-density shadows with large, distorted feeding arteries, which were obviously intensified on enhanced film. Eight patients received tumor excision and/or chemotherapy with vincristine, six of whom survived. Pathological results confirmed the diagnosis of kaposiform hemangioendothelioma. Total five patients died from extensive hemorrhage and subsequent multiple organ failure. Conclusion Chinese children with KMP have typical symptoms. Kaposiform hemangioendothelioma may be the most common vascular tumor associated with KMP. Surgery combined with chemotherapy is an effective approach for KMP.