上气道Rosai-Dorfman病临床及病理特点的回顾性研究  

Upper airway Rosai-Dorfman disease:a retrospective study on clinical and pathological features

作　　者：何雨蓉 钟琦[1] 房居高[1] 张洋[1] 侯丽珍[1] 陈晓红[1] 王元 王哓艳 HE Yurong;ZHONG Qi;FANG Jugao;ZHANG Yang;HOU Lizhen;CHEN Xiaohong;WANG Yuan;WANG Xiaoyan(Department of Otolaryngology Head and Neck Surgery,Beijing Tongren Hospital,Capital Medical University,Beijing,100730,China)

机构地区：[1]首都医科大学附属北京同仁医院耳鼻咽喉头颈外科,北京100730

出　　处：《中国耳鼻咽喉头颈外科》2022年第2期69-74,共6页Chinese Archives of Otolaryngology-Head and Neck Surgery

摘　　要：目的 回顾性分析上气道Rosai-Dorfman病(RDD)的临床特点、组织病理学特征、治疗及预后。方法2011年3月~2021年4月首都医科大学附属北京同仁医院收治21例上气道RDD患者的临床病例资料进行回顾性总结分析,分为鼻腔鼻窦组16例,喉气管组5例,治疗以手术切除为主(18例,85.7%)。结果 喉气管组以呼吸困难(80%)多见,鼻腔鼻窦组以鼻塞(81.3%)和鼻部肿胀(43.8%)多见。病理表现为大量胞浆透亮的组织细胞、淋巴细胞和浆细胞浸润,10例镜下表现出典型“伸入现象”。实验室检查中红细胞沉降率(ESR)升高者为55.6%,上气道RDD术后复发率为50%。结论 上气道RDD是临床上极为罕见的以淋巴组织增生为主要特点免疫相关性疾病。最常发生于鼻腔鼻窦,多见于中年女性。患者多因局部占位或梗阻症状就诊,ESR升高可能有提示作用,该病诊断需建立在典型的组织病理学特征和免疫组化基础上,特别是S-100、CD68及IgG4阳性。治疗以手术或手术联合激素/化疗为主,术后复发率较高,复发后可再行手术或内科系统治疗。OBJECTIVE To retrospectively investigate the clinical features,histopathological features,treatment and prognosis of Rosai-Dorfman disease(RDD) with upper airway involvement.METHODS Twenty-one clinical cases of RDD with upper airway involvement,diagnosed or treated in Beijing Tongren Hospital Affiliated to Capital Medical University between 2011 and 2021,were retrospectively summarized and analyzed.There were 16 cases in the nasal cavity and paranasal sinus and 5 cases in the laryngotracheal region,the main treatment was surgical resection(18 cases,85.7%).RESULTS RDD patients inlaryngotracheal region presented with dyspnea(4/5,80%) mostly;The main symptoms in patients with nasal cavity and paranasal sinus lesions were nasal congestion(13/16,81.3%) and nasal swelling(7/16,43.8%).The striking histopathological manifestion was the proliferation and infiltration of a large number of tissue cells with bright cytoplasm,lymphocytes and plasma cells.Ten cases presented with a typical ‘emperipolesis’ under microscope.Five of nine cases(55.6%) had increased erythrocyte sedimentation rate(ESR) in the laboratory examination.The recurrence rate of upper airway RDD was 50%.CONCLUSION Upper airway RDD is a rare clinical immune-related disease characterized by lymphoid tissue hyperplasia.It most often occurred in the nasal cavity and paranasal sinuses,mostly in middle-aged women.Most of the patients went to the hospital due to local space occupying or obstructive symptoms,and the increase of ESR may be suggestive.The diagnosis of the disease should be based on typical histopathological features and immunohistochemistry,especially the positivity of S-100,CD68 and IgG4.The treatment is mainly surgery or surgery combined with hormone/chemotherapy,most patients could be treated with minimally invasive surgery.The postoperative recurrence rate is high,surgery or systemic therapy might be performed again after recurrence.

关 键 词：头颈部肿瘤 淋巴结病 ROSAI-DORFMAN病 窦组织细胞增生伴巨大淋巴结病 组织细胞增生症 上气道 

分 类 号：R551.2[医药卫生—血液循环系统疾病]