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 treatment can quickly relieve clinical symptoms. Although RDD is a benign lesion, extranodal RDD may have serious consequences because of its location; therefore, it should be paid attention to in the clinic.

Key words: Rosai-Dorfman disease, Sinus histiocytosis with massive lymphadenopathy, Immunohistochemistry, Diagnose, Therapy