窦组织细胞增生伴巨大淋巴结病诊疗进展

Sinus histiocytosis with massive lymphadenopathy

窦组织细胞增生伴巨大淋巴结病（sinus histiocytosis with massive lymphadenopathy,SHML）又称为Rosai—Dorfman病，是一种病因不明，罕见的良性淋巴组织增生性疾病，好发于儿童及青少年。因其累及部位不同而临床表现多样，无明确实验室指标支持该病，且属于儿科少见病，在儿科临床极易误诊和漏诊，本文对其诊断及治疗方法等进行归纳总结，以提高对该疾病的认识。

Sinus histiocytosis with massive lymphadenopathy（SHML） is also called Rosai-Dorfman disease. It is a kind of benign lymphoid tissue proliferative diseases with unknown etiology. SHML appeared mostly in children and adolescent. It has diverse clinical manifestations accompanied with multiple organ injury, and no clear laboratory indicators could support the disease, being a rare disease in pediatrics, easy to miss diagnosis. This article reviewed the latest progress on diagnostis and treatment of SHML, to improve the understanding of the disease.