Chang Yu-Chien, Tsai Ming-Hsui, Chen Chi-Long, Tsai Chon-Haw, Lee Alex Ying-Shyuan
Department of Otolaryngology, China Medical College Hospital, Taichung, Taiwan.
Am J Otolaryngol. 2003 May-Jun;24(3):183-6. doi: 10.1016/s0196-0709(03)00002-4.
Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) is a rare but distinctive entity. It was initially described as a nodal-based disease but later found to affect a variety of extranodal sites. The diagnosis of Rosai-Dorfman disease is based on the unique histologic changes that are characterized by proliferation of S-100 protein-positive histiocytes, the presence of emperipolesis, and a mixture of prominent lymphoplasmacytic infiltrates. The etiology of Rosai-Dorfman disease is unknown. It usually runs a benign clinic course and may resolve spontaneously, but cases running protracted courses have also been reported. We report an unusual case of recurrent extranodal Rosai-Dorfman disease in nasal and paranasal cavities with intracranial involvement surviving for more than 9 years.
伴巨大淋巴结病的窦组织细胞增生症（罗萨伊 - 多夫曼病）是一种罕见但独特的疾病。它最初被描述为一种基于淋巴结的疾病，但后来发现可累及多种结外部位。罗萨伊 - 多夫曼病的诊断基于独特的组织学改变，其特征为S - 100蛋白阳性组织细胞增生、存在血细胞吞噬现象以及显著的淋巴浆细胞浸润混合存在。罗萨伊 - 多夫曼病的病因尚不清楚。它通常呈良性临床病程，可能会自发缓解，但也有报道称存在病程迁延的病例。我们报告了一例罕见的鼻腔和鼻窦复发性结外罗萨伊 - 多夫曼病伴颅内受累且存活超过9年的病例。
