CAJ | 학술논문

目的：探讨淋巴结外窦组织细胞增生伴巨大淋巴结病（ SHML，Rosai-Dorfman病）的病理诊断，免疫组化特点及鉴别诊断。方法对3例淋巴结外SHML患者进行HE、免疫组化染色观察，并复习相关文献。结果3例患者男性1例，女性2例，年龄38～72岁。其中一例为多发病变，位于胸部及腹壁软组织。另两例为单发病变，分别位于右上肢软组织及右侧胫骨下段。组织学显示病变内大量组织细胞、淋巴细胞、浆细胞浸润，形成染色深浅不一的结节样区域。组织细胞体积大，胞浆丰富，核呈泡状，可见小核仁，在组织细胞的胞浆内，可见淋巴细胞吞噬现象。免疫组织化学染色示该组织细胞表达S-100蛋白与CD68。结论淋巴结外SHML是一种罕见的组织细胞增生性病变，在病理诊断方面，易误诊、漏诊，明确病理组织学特点在鉴别诊断中有重要意义。
목적：탐토림파결외두조직세포증생반거대림파결병（ SHML，Rosai-Dorfman병）적병리진단，면역조화특점급감별진단。방법대3례림파결외SHML환자진행HE、면역조화염색관찰，병복습상관문헌。결과3례환자남성1례，녀성2례，년령38～72세。기중일례위다발병변，위우흉부급복벽연조직。령량례위단발병변，분별위우우상지연조직급우측경골하단。조직학현시병변내대량조직세포、림파세포、장세포침윤，형성염색심천불일적결절양구역。조직세포체적대，포장봉부，핵정포상，가견소핵인，재조직세포적포장내，가견림파세포탄서현상。면역조직화학염색시해조직세포표체S-100단백여CD68。결론림파결외SHML시일충한견적조직세포증생성병변，재병리진단방면，역오진、루진，명학병리조직학특점재감별진단중유중요의의。
Objective To study the pathological diagnosis ,immunohistochemical features and differential diagnosis of extranodal Rosai -Dorfman disease ( RDD) .Methods Three cases of extranodal RDD were investigated by HE staining and immunohistochemical stai -ning,and related literatures were reviewed .Results Among the 3 cases studied,there were 1 male and 2 females.The age of patients ranged from 38 to 72 years old.The lesion arose in the chest and abdominal wall (1 case),right arm(1 case) or right tibia(1 case).Mi-croscopically,the lesions were characterized by variable numbers of large histiocytes with emperipolesis ,accompanied by infiltration of lymphocytes and plasma cells .Lymphocytes and plasma cell formed irregular nodal structure with light and dark appearance under mi -croscope.The large histocytes had abundant cytoplasm , a vesicular nucleus and a small basophilic nucleolus .Immunohistochemistry study showed that the histiocytes expressed S-100 protein and CD68.Conclusions RDD of tone and soft tissue is very rare and liable to be misdiagnosed in pathologic diagnosis .The histopathologic features are therefore of significance in differential diagnosis .