结外Rosai-Dorfman病的相关临床病理特征及文献复习  

The clinicopathological characteristics and literature review of extranodal rosai-dorfman disease

作　　者：张亚男 潘国庆[2] 李睿[2] 王智园 施意 刘士岳 ZHANG Yanan;PAN Guoqing;LI Rui;WANG Zhiyuan;SHI Yi;LIU Shiyue(Department of Pathology,Longling People's Hospital,Banshan,Yunnan,China,678300;Department of Pathology,the First Affiliated Hospital of Kunming Medical University,Kummimg,Yunnan,China,653200;Department of Pathology,Baoshan People's Hospital,Banshan,Yunnan,China,678300)

机构地区：[1]保山市龙陵县人民医院病理科,云南保山678300 [2]昆明医科大学第一附属医院病理科,云南昆明653200 [3]保山市人民医院病理科,云南保山678300

出　　处：《分子诊断与治疗杂志》2022年第6期1068-1071,共4页Journal of Molecular Diagnostics and Therapy

基　　金：国家自然科学基金项目(81660413);云南省教育厅科学研究基金项目(2018JS201);云南省教育厅科学研究基金项目(2019J1239)。

摘　　要：目的 观察18例结外Rosai-Dorfman病(RDD)的临床病理特征并结合文献探讨其病理诊断以提高病理医生及临床医生对此病的认识。方法 收集2013年8月至2019年12月昆明医科大学第一附属医院病理科18例结外Rosai-Dorfman病患者的临床资料,描述病例的显微镜下特点,并对所有病例进行CD68、S-100及CD1a蛋白免疫组化染色。结果 RDD可以发生于人体大部分器官且均为首发部位。显微镜下可见由组织细胞和混合性炎症细胞组成交替分布的淡染带和深染带,伴有特征性的窦组织细胞(Rosai-Dorfman细胞)增生;免疫组化染色组织细胞均弥漫强阳性表达S-100、CD68,不表达CD1a。经过后期电话随访所有患者均健在且无复发。结论 结外RDD的发生与性别无关,所有年龄段均可发病,全身上下所有系统均可发生,病理学诊断依据其典型的显微镜下特征及免疫组织化学结果,手术切除可治愈。Objective To observe the clinicopathological features of 18 cases of extranodal RDD and explore its pathological diagnosis based on the literature,so as to improve the understanding of pathologists and clinicians. Methods Collected in August 2013 to December 2019,the first affiliated hospital of kunming medical university department of 18 cases of extranodal RDD patients clinical data, describe the microscopic characteristics and through immunohistochemical staining method for CD68、S-100 and CD1a immunohistochemical staining. Results RDD could occur in mostorgans of the human body and all of them were the first site.Microscopic examination showed alternately light and dark staining zones composed of histrocytes and mixed inflammatory cells,accompanied by characteristic proliferation of sinus histrocytes(Rosai-Dorfman cells). Immunohistochemical staining showed strong diffusely positive expression of S-100 and CD68protein,but no expression of CD1a protein. After telephone follow-up,all patients were alive without recurrence. Conclusions The occurrence of extranodal RDD is independent of gender,but can occur in all ages and all systems of the body. The pathological diagnosis is based on its typical microscopic characteristics and immunohistochemical results,and surgical resection can be cured.

关 键 词：ROSAI-DORFMAN病 CD68蛋白 S-100蛋白 

分 类 号：R551.2[医药卫生—血液循环系统疾病]