颌面部血友病假肿瘤的临床特点及疗效观察  

A retrospective study of hemophilic pseudotumor in maxillofacial region

作　　者：于国霞[1] 陈振萍[2] 王新刚[1] 陈卓 刘颖[1] 刘国青[2] 吴润晖[2] Yu Guoxia;Chen Zhenping;Wang Xingang;Chen Zhuo;Liu Ying;Liu Guoqing;Wu Runhui(National Center for Children′s Health&Department of Stomatology,Beijing Children′s Hospital,Capital Medical University,Beijing 100045,China;National Center for Children′s Health&Department of Hematology,Beijing Children′s Hospital,Capital Medical University,Beijing 100045,China)

机构地区：[1]国家儿童医学中心,首都医科大学附属北京儿童医院口腔科,100045 [2]国家儿童医学中心,首都医科大学附属北京儿童医院血液科,100045

出　　处：《中华口腔医学杂志》2021年第8期785-790,共6页Chinese Journal of Stomatology

基　　金：北京市科学技术委员会首都临床特色应用研究基金(Z181100001718182);首都卫生发展科研专项(首发2018-2-2094)。

摘　　要：目的总结分析颌面部血友病假肿瘤的临床特点、诊疗及预后,为医师提供治疗选择参考。方法收集首都医科大学附属北京儿童医院口腔颌面外科2009年1月至2019年1月收治的14例颌面部血友病假肿瘤患者,其中2例失访,最终完成随访并纳入研究者12例,均为男性,年龄13个月~10岁。回顾患者病史包括家族史、外伤史及院外治疗史,分析患者的实验室检查和影像学检查资料,以及诊疗过程和治疗方案等,随访了解患者的预后,随访时间为13个月~10年。12例患者均经过保守因子替代治疗。血友病A11例,血友病B1例,其中轻型5例,中间型5例,重型2例;其中就诊前确诊血友病仅1例,9病例被误诊为肿瘤,1例误诊为血管瘤,1例误诊为骨髓炎,仅有3例有明确外伤史。结果共12例完成随访病例,所有病例均先采用保守因子替代治疗,其中10例(10/12)有效,其中保守因子替代治疗8例(8/12)治愈,1例保守因子替代治疗后尚遗留皮肤软组织瘘管,1例保守因子替代治疗8个月停药后半年复发再次替代治疗。2例效果不佳选择手术加围术期替代治疗治愈。结论颌面部血友病假肿瘤误诊率高,保守因子替代治疗大部分患者可以取得较好的效果,可作为首选治疗方式,若保守因子替代治疗效果不佳,进行手术治疗也是相对安全的选择。Objective To provide clinical references for the diagnosis and treatment of hemophilic pseudotumor(HPT)in maxillofacial region.Methods Fourteen cases of HPT in maxillofacial region from the Department of Stomatology,Beijing Children′s Hospital from Jan 2009 to Jan 2019 were collected.Two cases were lost for follow-up and 12 patient,all boys,were finally followed up and included in the study.The patients aged from 13 months to 10 years old.The medical history,clinic manefestitions and the features of the radiology examination were recorded.The patients were treated by using replacement treatment first.If the conservative treatment was not effective,the patients then received operation combined with pereoperation replacement thearapy.The patients were followed up for 13 months to 10 years.There were 11 cases of hemophilia A,and 1 case of hemophilia B.Two cases were severe type,the others(10/12)were mild and moderate types.Only 1 case was diagnosed as hemophilia initially.Nine cases(9/12)were misdiagnosed as malignant tumors,1 case was misdiagnosed as osteomyelitis and 1 case was misdiagnosed as hemangioma.Only 3 cases had identified history of trauma before.Results All cases were treated with replacement therapy first,among which 10 cases were effective,8 cases were cured by conservative therapy,1 case had residual soft tissue fistula after conservative treatment and 1 case recurrented after conservative treatment for 8 months.Two patients with poor efficacy to the replacement treatment were performed operations and finally were cured.Conclusions The misdiagnosis rate of HPT in maxillofacial region was high.The conservative factor replacement therapy could achieve good results in most children and could be used as the preferred treatment.If the conservative treatment was not effective,the surgical treatment was also a safe option.

关 键 词：血友病 颌面部假肿瘤 替代治疗 保守治疗 

分 类 号：R554.1[医药卫生—血液循环系统疾病]