窦组织增生症伴巨大淋巴结病1例报告并文献复习

目的 提高对窦组织增生症伴巨大淋巴结病(Rosai-Dorfman病)的临床表现和病理诊断的认识.方法 报道1例经淋巴结活检确诊Rosai-Dorfman病患者的临床资料并结合文献进行讨论.结果 患者肺CT平扫示双侧肺野内可见多发、弥漫性分布的大小不等的结节影,纵隔内可见多组肿大淋巴结影.淋巴结病理可见组织细胞、浆细胞和淋巴细胞并可见吞噬淋巴细胞现象,免疫组化S-100及CD68阳性.该病临床表现多样,易误诊.结论 Rosai-Dorfman病累及气道是罕见的淋巴结外病变,治疗以综合治疗为主,治疗后随访.

Rosai-Dorfman disease:a case report and literature review

Objective To enhance the understanding of the clinical manifestations and pathological characteristics of sinus histioeytosis with massive lymphadenopathy{Rosai Dorfman disease）. Methods To discuss the clinical data of a patient diagnosed with Rosai Dorfman disease by lymph node biopsy,and review the related literatures. Results Chest CT scan showed bilateral multiple and diffuse nodules different in size,and obvious enlargement of mediastinal lymph nodes could be seen; Pathological examination of lymph nodes revealed numberous histiocytes, plasma cells and lymphocytes,and phagocytosed intact lymphoeytes. Immunohistochemical staining positive for S100 protein and CD 68 was suggestive of the diagnosis. But misdiagnosis and mistreatment of it was frequent because of multiplex clinical manifestation. Conclusions Rosai Dorfman disease involving the airway is a rare disease. Comprehensive treatment is the optimal treatment,follow-up is essential after treatment.