Noordzij Walter, Weernink Erna E M, van Imhoff Gustaaf W, Kluin Philip M, de Haan L D Dick
Scheper Ziekenhuis, afd. Interne Geneeskunde, Emmen, the Netherlands.
Ned Tijdschr Geneeskd. 2011;155(27):A3176.
Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease (RDD)) is a rare entity with pathological accumulation of specific, activated macrophages, mainly in cervical lymph nodes. However, in half of patients its primary presentation is extranodal. Its aetiology is unclear; several clues point in an immunological direction. Common clinical presentation is massive cervical lymphadenopathy, but nearly half of all patients suffer from extranodal manifestation at very diverse localizations. Diagnosis is based on histopathological findings: emperipolesis and S-100 positive histiocytes. Taking into account its benign character, caution in implementing therapy is recommended. Therapeutic options are limited to specific situations only.
Two patients with known RDD presented at our outpatient clinic. A 75-year-old woman with two tumours in her right breast and an 83-year-old man with constipation - most likely due to a kidney tumour. Microscopic findings finally confirmed extranodal manifestation of RDD.
Extranodal manifestations of RDD present in various forms and on history taking, physical examination and radiological imaging may resemble malignancies.
伴巨大淋巴结病的窦组织细胞增生症（罗萨伊 - 多夫曼病（RDD））是一种罕见疾病，其特征为特定的活化巨噬细胞病理性积聚，主要累及颈部淋巴结。然而，半数患者的首发表现为结外病变。其病因尚不清楚；多条线索指向免疫方向。常见临床表现为颈部巨大淋巴结病，但几乎一半的患者在非常不同的部位出现结外表现。诊断基于组织病理学发现：吞噬现象和S - 100阳性组织细胞。鉴于其良性特征，建议在实施治疗时谨慎。治疗选择仅限于特定情况。
两名已知患有RDD的患者前来我们门诊就诊。一名75岁女性右乳有两个肿瘤，一名83岁男性便秘——很可能是由肾肿瘤引起。显微镜检查结果最终证实为RDD的结外表现。
RDD的结外表现形式多样，通过病史采集、体格检查和影像学检查可能类似恶性肿瘤。
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