CAJ | 학술논문

目的：探讨淋巴结外Rosai-Dorfman病的临床病理特点、免疫组化表型及鉴别诊断。方法分析6例淋巴结外Rosai-Dorfman病患者的临床表现，并对切除标本进行常规HE病理学检查，采用PV-6000法进行免疫组织化学标记。结果6例Rosai-Dorfman病患者中有3例肿块位于胸壁，1例位于鼻粘膜、中鼻甲，1例位于胫骨，1例位于股骨。镜下示病变组织由淡染区和深染区构成的结节状病灶，淡染区为增生的组织细胞，细胞大，胞质丰富，部分细胞胞质内见吞噬的淋巴细胞、浆细胞，深染区为淋巴细胞、浆细胞等构成，部分病例病变区夹杂有嗜酸性粒细胞、中性粒细胞等炎细胞。免疫组化结果示增生的组织细胞S-100阳性，CD68部分阳性或阴性，CD1α阴性。结论淋巴结外Rosai-Dorfman病是1种少见的组织细胞病变，有其独特的病理学形态和免疫组化表型，对增生组织细胞本质的认识是正确诊断及鉴别诊断的关键。
목적：탐토림파결외Rosai-Dorfman병적림상병리특점、면역조화표형급감별진단。방법분석6례림파결외Rosai-Dorfman병환자적림상표현，병대절제표본진행상규HE병이학검사，채용PV-6000법진행면역조직화학표기。결과6례Rosai-Dorfman병환자중유3례종괴위우흉벽，1례위우비점막、중비갑，1례위우경골，1례위우고골。경하시병변조직유담염구화심염구구성적결절상병조，담염구위증생적조직세포，세포대，포질봉부，부분세포포질내견탄서적림파세포、장세포，심염구위림파세포、장세포등구성，부분병례병변구협잡유기산성립세포、중성립세포등염세포。면역조화결과시증생적조직세포S-100양성，CD68부분양성혹음성，CD1α음성。결론림파결외Rosai-Dorfman병시1충소견적조직세포병변，유기독특적병이학형태화면역조화표형，대증생조직세포본질적인식시정학진단급감별진단적관건。
Objective To investigate the clinicopathological features,immunophenotype and differential diagnosis of ex-tranodal Rosai-Dorfman disease.Methods Clinical manifestation were analyzed and morphologic features and immunohistochem-ical staining by using immunohistochemistry of PV-6000 were studied in 6 cases extranodal Rosai-Dorfman disease.Results A-mong 6 cases extranodal Rosai-Dorfman disease,3 cases had lesions in chest,and other 3 cases located in nasal mucous、concha bullosa,tibia,femur,respectively.Histopathologic features was characterized by light-staining bands alternating with dark-staining bands.The light-staining bands were formed by aggregates of large histiocytes,and there were lymphocytes and plasma cells in some histiocytes’cytoplasm.The dark-staining bands were formed by lymphocytes and plasma cells,and neutrophils,eosinophils and other inflammatory cells.Immunohistochemical study showed that the histiocytes were strongly positive for S-100,and partly positive or negative for CD68,negative for CD1α.Conclusion Extranodal Rosai-Dorfman disease is a rare histiocytic proliferative disorder which has distinct morphologic feature and immunophenotype.Recognization of the large histiocytes is a key point for cor-rect diagnosis and differential diagnosis.