Remadi S, Anagnostopoulou I D, Jlidi R, Cox J N, Seemayer T A
Institut de Pathologie Clinique, CMU, Geneve, Switzerland.
Pathol Res Pract. 1996 Oct;192(10):1007-15. doi: 10.1016/s0344-0338(96)80042-2.
We describe two children with Rosai-Dorfman disease who presented with exophthalmos, leukocytosis, an elevated sedimentation rate and hypergammaglobulinemia. Both became blind as a result of this condition. One child had associated bilateral cervical lymphadenopathy. Investigation revealed involvement of the nasal fossae and retro-orbital spaces by tumoural masses histologically consistent with the diagnosis of sinus histiocytosis with massive lymphadenopathy (SHML). Immunohistochemical studies suggest that these histiocytes are activated macrophages derived from a phenotype hybrid between "professional" phagocytic cells and immune accessory cells, expressing lysosomal antigens, S-100 protein but rarely, CDla. An underlying immune dysfunction may be central to the pathogenesis of this disease.
我们描述了两名患有罗萨伊-多夫曼病的儿童，他们表现为眼球突出、白细胞增多、血沉加快和高球蛋白血症。两人均因此病失明。其中一名儿童伴有双侧颈部淋巴结病。检查发现鼻腔和眶后间隙有肿瘤性肿块，组织学上与伴有巨大淋巴结病的窦组织细胞增生症（SHML）的诊断相符。免疫组化研究表明，这些组织细胞是活化的巨噬细胞，源自“专业”吞噬细胞和免疫辅助细胞之间的表型杂交，表达溶酶体抗原、S-100蛋白，但很少表达CD1a。潜在的免疫功能障碍可能是该疾病发病机制的核心。
