Pradhananga Rabindra Bhakta, Dangol Kripa, Shrestha Anjan, Baskota Dharma Kanta
Department of ENT-Head and Neck Surgery, Tribhuvan University Teaching Hospital, Kathmandu, Nepal.
Department of Pathology, Tribhuvan University Teaching Hospital, Kathmandu, Nepal.
Int Arch Otorhinolaryngol. 2014 Oct;18(4):406-8. doi: 10.1055/s-0034-1387163. Epub 2014 Aug 25.
Introduction Rosai-Dorfman disease (RDD) is a rare histiocytic proliferative disorder of unknown etiology. Usually it presents with massive painless cervical lymph node enlargement. Histologically, it shows proliferation of distinctive histiocytic cells that demonstrate emperipolesis in the background of a mixed inflammatory infiltrates. Immunohistochemically, the cells are positive for markers such as CD68 and S100. Objective To report a case of a 12-year-old patient with multiple sites of cervical lymphadenitis, which was diagnosed as RDD histopathologically as well as immunohistologically. Resumed Report A 12-year-old girl presented with multiple painless sites of cervical lymphadenitis without any systemic and other ear, nose, and throat manifestations. The biopsy report of the lymph node showed dilatation of the sinuses, filled with histiocytes having foamy cytoplasm. Many of the histiocytes were engulfing mature lymphocytes. The sinus histiocytes were strongly positive for S-100 protein. Conclusion RDD must be considered in the differential diagnosis of massive or multiple lymphadenopathies.
引言 罗萨伊-多夫曼病（RDD）是一种病因不明的罕见组织细胞增生性疾病。通常表现为颈部淋巴结无痛性肿大。组织学上，它显示出独特的组织细胞增生，这些细胞在混合性炎症浸润背景下呈现吞噬现象。免疫组织化学检查显示，这些细胞对CD68和S100等标志物呈阳性反应。目的 报告1例12岁患有多处颈部淋巴结炎的患者，经组织病理学及免疫组织化学检查确诊为RDD。病例报告 一名12岁女孩出现多处颈部无痛性淋巴结炎，无任何全身及耳鼻咽喉相关表现。淋巴结活检报告显示窦腔扩张，充满细胞质呈泡沫状的组织细胞。许多组织细胞吞噬成熟淋巴细胞。窦组织细胞S-100蛋白呈强阳性。结论 在鉴别诊断大量或多处淋巴结病时必须考虑RDD。
