Wang W S, Liu J H, Yang C F, Yang A H, Chen P M
Department of Medicine, Veterans General Hospital-Taipei and National Yang-Ming University School of Medicine, Taiwan, R.O.C.
Zhonghua Yi Xue Za Zhi (Taipei). 1996 Oct;58(4):281-5.
A 28-year-old woman was admitted to our hospital with the chief complaint of progressive gingival swelling with easy bleeding and loosening of teeth for about two years. The liver and spleen were not enlarged and there were no lymphadenopathy. The hemogram was normal. The skull X-ray showed floating teeth. CT scan of face showed destructive bony lesions over maxilla and left mandible with adjacent soft tissue swelling. Pathologic examination of the gingiva revealed that the oral mucosal tissue was heavily infiltrated with histiocyte-like Langerhans' cells. The Langerhans' cells showed positive immunostain for S-100 protein. Under electron microscope, ultrastructure of the Langerhans' cells revealed typical intracytoplasmic tennnis racquet-shaped structure with a central zipper-like striation (Birbeck's granules). Langerhans' cell histiocytosis was diagnosed. She received rdiotherapy with a total dose of 1,000 cGy in fractions and oral chemotherapeutic drugs. Painful gingival swelling subsided gradually. She was followed at our OPD for the past 10 months and there was no evidence of local recurrence. Langerhans' cell histiocytosis is a rare disease. The relevant literature about its distinct pathologic features, clinical course and treatment is reviewed.
一名28岁女性因渐进性牙龈肿胀、易出血和牙齿松动约两年为主诉入院。肝脏和脾脏未肿大，无淋巴结病。血常规正常。颅骨X线显示牙齿漂浮。面部CT扫描显示上颌骨和左下颌骨有骨质破坏病变，伴有相邻软组织肿胀。牙龈病理检查显示口腔黏膜组织有大量组织细胞样朗格汉斯细胞浸润。朗格汉斯细胞S-100蛋白免疫染色呈阳性。在电子显微镜下，朗格汉斯细胞的超微结构显示典型的胞质内网球拍状结构，中央有拉链样条纹（伯贝克颗粒）。诊断为朗格汉斯细胞组织细胞增多症。她接受了分次总量为1000 cGy的放射治疗和口服化疗药物。疼痛性牙龈肿胀逐渐消退。在过去10个月里她在我们的门诊接受随访，没有局部复发的迹象。朗格汉斯细胞组织细胞增多症是一种罕见疾病。本文回顾了有关其独特病理特征、临床病程和治疗的相关文献。
