CAJ | 학술논문

目的提高对颌面部血友病假性肿瘤的诊断意识并探讨该病的外科治疗方法.方法回顾5年来4例颌面部血友病假性肿瘤的诊断及治疗过程,并坚持随访2年以上,分析其特点及预后.结果 4例患者治疗前均误诊,并有出血史.1例在无替代疗法下行病灶活检致出血不止；1例无诱因牙龈出血显著,常规止血措施效果不佳,伴出血性贫血.实验室检查4例均存在因子Ⅷ缺乏,确诊为血友病A型.经内科替代治疗后手术治疗,围手术期加强因子Ⅷ活度监测,术后均无严重出血,伤口一期愈合,随访均未见复发.病理检查未见肿瘤细胞,明确血友病假性肿瘤诊断.结论颌面部血友病假性肿瘤易误诊,可伴有大出血等严重并发症,临床要给予足够的认识,需结合实验室检查及易出血等病史给予确诊,辅以内科替代疗法保障下的手术治疗可以减少出血,防止假性肿瘤进行性增大所致畸形.
목적 제고대합면부혈우병가성종류적진단의식병탐토해병적외과치료방법.방법 회고5년래4례합면부혈우병가성종류적진단급치료과정,병견지수방2년이상,분석기특점급예후.결과 4례환자치료전균오진,병유출혈사.1례재무체대요법하행병조활검치출혈불지；1례무유인아간출혈현저,상규지혈조시효과불가,반출혈성빈혈.실험실검사4례균존재인자Ⅷ결핍,학진위혈우병A형.경내과체대치료후수술치료,위수술기가강인자Ⅷ활도감측,술후균무엄중출혈,상구일기유합,수방균미견복발.병리검사미견종류세포,명학혈우병가성종류진단.결론 합면부혈우병가성종류역오진,가반유대출혈등엄중병발증,림상요급여족구적인식,수결합실험실검사급역출혈등병사급여학진,보이내과체대요법보장하적수술치료가이감소출혈,방지가성종류진행성증대소치기형.
Objective To present 4 cases of hemophilic pseudotumor and to investigate the treatment outcome.Methods Four cases of hemophilic pseudotumor were reviewed.The patients were treated by internal medicine combined with surgery and followed up for two years.The feature and diagnosis of the disease were analyzed and the treatment outcome was evaluated.Results All of the 4 cases were misdiagnosed with a history of bleeding before operation.One patient was bleeding after biopsy without replacement therapy.One patient was presented with gingival bleeding and anaemia.The final diagnosis of hemophilia A in all the 4 cases was confirmed by the blood test(Ⅷ deficiency).After infusion of factor Ⅷ,operation was performed on all cases.There was no recurrence after two years of follow-up.Conclusions It should be aware of the rarely encountered disease which is prone to be misdiagnosed.Under factor-deficient replacement therapy,surgical management is the most effective way in preventing from bleeding and avoiding progressive expanding of pesudotumor.