Rosai-Dorfman病13例临床病理分析  

Clinicopathological analysis of 13 cases of Rosai-Dorfman Disease

作　　者：向丽萍 何惠华[1] 袁静萍[1] 刘琳[1] 饶洁[1] Xiang Liping;He Huihua;Yuan Jingping;Liu Lin;Rao Jie(Department of Pathology,Renmin Hospital of Wuhan University,Wuhan 430060)

机构地区：[1]武汉大学人民医院病理科,武汉430060

出　　处：《中国组织化学与细胞化学杂志》2023年第1期77-80,共4页Chinese Journal of Histochemistry and Cytochemistry

基　　金：湖北省卫生健康科研基金资助(WJ2021M151)。

摘　　要：目的 探讨Rosai-Dorfman病(Rosai-Dorfman Disease,RDD)的临床病理特征、诊断及鉴别诊断,并查找相关文献对该疾病进行总结复习。方法 回顾性研究武汉大学人民医院2016-2021年诊断为Rosai-Dorfman病的病例,所有病例均由2位高年资诊断医师复阅。结果 13例患者,年龄16~71岁,男性4例,女性9例,男女比例1∶2.25。9例为结外RDD,3例为混合型RDD,1例为肿瘤形成相关型。发生部位:鼻腔鼻窦5例,其中2例伴有颈部淋巴结无痛性肿大,皮肤型RDD 3例,降主动脉、颌下腺、回盲瓣、眼眶、肺各1例,其中发生于回盲瓣者并发非霍奇金淋巴瘤,发生颌下腺者伴有颈部淋巴结肿大。临床表现因发生部位不同而表现不一。镜下形态大致相同,可见大量组织细胞、淋巴细胞以及浆细胞,呈现交替排列的暗区和亮区,高倍镜下可见“伸入现象”,即组织细胞内含有完整的淋巴细胞,大多病例可伴有明显的纤维化。免疫组织化学染色显示S-100、CD68阳性,CD1α阴性。结论 Rosai-Dorfman病发病率低,发病年龄广,女性患者较多见,易发生于鼻腔鼻窦,临床表现及影像学不具有特征性,诊断主要依靠镜下特征及免疫组织化学。Objective To investigate the clinicopathological features, diagnosis, and differential diagnosis of Rosai-Dorfman disease (RDD), and to review the relevant literature. Methods A retrospective study was conducted on 13 cases of Rosai-Dorfman disease diagnosed in Renmin Hospital of Wuhan University from 2016 to 2021. All cases were reviewed by two senior diagnosticians. Results 13 patients were 16-71 years old, including 4 males and 9 females, with a male to female ratio of 1:2.25. Among the 13 cases, 9 cases were extra-nodal RDD, 3 cases were mixed RDD, and 1 case was tumor formation related RDD. There were 5 cases of RDD oc­curred in nasal cavity and paranasal sinuses (2 cases were accompanied by painless swelling of neck lymph nodes), 3 cases of cutaneous type RDD, RDD occurred in descending aorta, submandibular gland, ileocecal valve, orbit, and lung for one case each. Among them, those who occurred in ileocecal valve were complicated with non-Hodgkin’s lymphoma, and those who occurred in submandibular gland were accompanied by swelling of neck lymph nodes. Clinical manifestations vary according to the location of occurrence. Under the microscope, the morphology is roughly the same. A large number of histiocytes, lymphocytes and plasma cells can be seen, showing alternating dark areas and bright areas. The emperipolesis (intracytoplasmic lymphocytes) was observed at higher magnification. And most cases can be accompanied by obvious fibrosis. Immunohistochemical staining showed histiocytes were positive for S-100 and CD68 , negative for CD1-α. Conclusion The incidence rate of Rosai-Dorfman disease is low, the age of onset is wide, female patients are more common, it is easy to occur in nasal cavity and paranasal sinuses, the clinical manifestations and imaging are not characteris­tic, and the diagnosis mainly depends on microscopic characteristics and immunohistochemistry.

关 键 词：ROSAI-DORFMAN病 病理特征 结外型 组织细胞 

分 类 号：R593.2[医药卫生—内科学]