双颈部及鼻咽部Rosai-Dorfman病1例相关病例回顾
Rosai-Dorfman disease of the bilateral neck and nasopharynx:a case report and review of related cases
李亚茹 1许贞菊 1王效军 1付涛 1袁玥 1吴策1
作者信息
- 1. 青岛大学附属医院 耳鼻咽喉头颈外科,山东 青岛 266000
- 折叠
摘要
目的 探讨罗道病(rosai-dorfman disease,RDD)病因、临床表现及诊断依据,并总结其治疗经验.方法 回顾性分析 1例RDD患者的临床资料并总结青岛大学附属医院近 10 年来确诊为RDD患者的住院资料.结果 通过检索青岛大学附属医院病历系统,排除 3 条病理结果为非RDD的患者,共筛选出6 例病理结果为RDD的患者,加上本例患者共7 例.男女比例为4∶3,16～65 岁,累及皮肤2 例(2/7),颅内1 例(1/7),气管1 例(1/7),颈部淋巴结2 例(2/7),鼻腔鼻窦、腮腺、眼眶、腋下及颈淋巴结 1 例(1/7),其免疫组化结果(除累及气管患者无本院结果):S100(+)100%、CD68(+)83.3%、CD163(+)80%、CD1a(-)100%(仅 3 例患者行CD1a检测),且上述患者均行手术治疗,术后临床症状缓解.结论 经典RDD常表现为双侧颈淋巴结肿大,可伴有发热、盗汗、疲乏和体质量减轻,结外RDD及混合型RDD临床上并不多见,但其容易影响重要器官的功能,造成严重后果,且其诊断较为困难,需要与其他疾病进行鉴别诊断.目前RDD尚无统一治疗标准,对于出现临床症状的患者,手术治疗可迅速缓解临床症状.RDD虽为良性病变,但结外RDD可因其发生部位产生严重后果,因此临床上应加以重视.
Abstract
Objective In order to explore the etiology,clinical manifestations,diagnostic basis,and treatment experience of Rosai-Dorfman disease(RDD).Methods The clinical data of a patient with RDD were retrospectively analyzed,and the hospitalization data of patients diagnosed with RDD in our hospital in the last 10 years were summarized.Results By searching the case system of our hospital,three patients with non-RDD pathological results were excluded,and six patients with RDD pathological results were screened out,plus seven patients in this case.The male-to-female ratio was 4:3,and the patients were aged 16-65 years.There were two cases(2/7)involving the skin,one case(1/7)involving the brain,one case(1/7)involving the trachea,two cases(2/7)involving the cervical lymph nodes,and one case(1/7)involving the nasal sinus,parotid gland,orbit,armpit,and cervical lymph nodes.Conclusion Classical RDD often manifests as bilateral cervical lymph node enlargement,which may be accompanied by fever,night sweats,fatigue,and weight loss.Extranodal RDD and mixed RDD are rare in clinical practice.However,as they affect the function of important organs,cause serious consequences,and have a difficult diagnosis,they need to be differentiated from other diseases.At present,there is no unified standard treatment for RDD.For patients with clinical symptoms,surgical treat-ment can quickly relieve clinical symptoms.Although RDD is a benign lesion,extranodal RDD may have serious consequences be-cause of its location;therefore,it should be paid attention to in the clinic.
关键词
rosai-dorfman病/窦组织细胞增生伴巨大淋巴结病/免疫组织化学/诊断/治疗Key words
Rosai-Dorfman disease/Sinus histiocytosis with massive lymphadenopathy/Immunohistochemistry/Diagnose/Therapy引用本文复制引用
基金项目
青岛大学附属医院临床医学+X科研项目(2020-094835)
出版年
2024
NETL
NSTL
万方数据