头颈部混合型Rosai-Dorfman病1例及文献分析  

Nodal and extranodal Rosai- Dorfman disease in head and neck:a case report with literature review

作　　者：梁珩[1] 周境 陈小华[1] 庄泽航 胡静[1] 刘习强[1] 

机构地区：[1]中山大学光华口腔医学院·附属口腔医院,广东省口腔医学重点实验室,广州510055

出　　处：《中华口腔医学研究杂志（电子版）》2015年第6期31-33,共3页Chinese Journal of Stomatological Research(Electronic Edition)

基　　金：国家自然科学基金(81372885;81172567);教育部新世纪优秀人才支持计划(NCET-11-0535);高校基本科研业务经费中山大学青年教师培育项目(12ykpy69)

摘　　要：目的探讨头颈部混合型Rosai-Dorfman病(RDD)的临床表现、组织病理学特征、诊断、治疗及预后。方法对1例混合型RDD行苏木精-伊红染色和免疫组织化学染色观察,对其进行随访并结合文献回顾分析。结果光镜下见大量淋巴细胞、浆细胞以及胞质苍白或呈嗜酸性淡染的特征性组织细胞,可见明显的淋巴吞噬现象(emperipolesis),免疫组化S-100和CD68染色阳性,CD1a阴性。本病的临床表现不典型,伴有颌面骨破坏吸收,容易误诊。予重组人粒细胞巨噬细胞刺激因子等治疗后病情控制。结论头颈部混合型RDD具有一定的组织病理学特征,但临床表现多样,诊断较困难。该病的治疗方法较多,效果不确定,应以保守治疗为主,密切随访。Objective To evaluate the pathological features, clinical manifestations, diagnosis, treatments and prognosis of Rosai-Dorfman disease （RDD） in head and neck. Methods HE staining and immunohistochemical analysis were performed in a case with both nodal and extranodal RDD in head and neck, follow-up data was collected and pertinent literatures were reviewed. Results Characteristic histiocytes with abundant pale or eosinophilic cytoplasm, and many lymphocytes and plasma cells were observed under light microscopy. A condition termed emperipolesis was found. Immunohistochemical staining was positive for both S-100 and CD68 but negative for CD1a. The clinical manifestation, along with destruction and resorption in maxillofacial bone, was atypical in this case and therefore apt to make a misdiagnosis. The patient shows partial remission on regular clinical and radiological follow-up followed immunotherapy. Conclusions Although RDD in head and neck has its specific pathological features, it presents differential clinical presentations and is hard to make an accurate diagnosis. Various treatments were used but none of them proved to be widely effective. Conservative treatment may be suitable for RDD in head and neck, and moreover, a long-term follow-up is indispensable.

关 键 词：ROSAI-DORFMAN病 组织细胞增多症 头颈部 诊断 

分 类 号：R597[医药卫生—内科学]