Juskevicius R, Finley J L
Department of Pathology, Brody School of Medicine, East Carolina University, Greenville, NC 27858-4354, USA.
Arch Pathol Lab Med. 2001 Oct;125(10):1348-50. doi: 10.5858/2001-125-1348-RDDOTP.
Rosai-Dorfman disease is a rare histiocytic proliferative disorder of unknown origin and a distinct clinicopathologic entity also known as sinus histiocytosis with massive lymphadenopathy. The disease can involve extranodal tissues and rarely can present as salivary gland enlargement without significant lymphadenopathy. Involvement of the extranodal head and neck sites appears to be more common in patients with immunologic abnormalities. The disease was first described in 4 patients in 1969, and with later descriptions of more patients, the disease was established as a well-defined clinicopathologic entity. The characteristic pathologic feature of this disease is proliferation of distinctive histiocytic cells that demonstrate emperipolesis in the background of a mixed inflammatory infiltrate, consisting of moderately abundant plasma cells and lymphocytes. Fine-needle aspiration biopsy can be helpful in establishing the correct diagnosis, since surgical treatment is not necessary other than obtaining tissue for definitive diagnosis. We describe cytologic, histopathologic, and immunohistochemical features of a case of Rosai-Dorfman disease that involved a major salivary gland without significant lymphadenopathy in a 48-year-old patient with systemic lupus erythematosus. We also briefly discuss possible causes and pathogenesis and review the literature.
罗萨伊-多夫曼病是一种起源不明的罕见组织细胞增生性疾病，是一种独特的临床病理实体，也称为伴有巨大淋巴结病的窦性组织细胞增生症。该疾病可累及结外组织，很少表现为唾液腺肿大而无明显淋巴结病。结外头颈部部位受累在免疫异常患者中似乎更为常见。该疾病于1969年首次在4例患者中被描述，随着后来更多患者的报道，该疾病被确立为一种明确的临床病理实体。该疾病的特征性病理特征是在由中等数量的浆细胞和淋巴细胞组成的混合性炎症浸润背景中，独特的组织细胞增生并表现出细胞吞噬现象。细针穿刺活检有助于确立正确诊断，因为除获取组织进行明确诊断外无需手术治疗。我们描述了1例48岁系统性红斑狼疮患者的罗萨伊-多夫曼病，该病例累及主要唾液腺且无明显淋巴结病，包括其细胞学、组织病理学和免疫组化特征。我们还简要讨论了可能的病因和发病机制并回顾了相关文献。
