title: Unusual life-threatening Rosai-Dorfman disease of the trachea: role of NF-κB.
abstract: Rosai-Dorfman disease (RDD) is a rare non-neoplastic histioproliferative disorder characterised by painless lymphadenopathy, low fever, high erythrocyte sedimentation rate, leucocytosis and hypergammaglobulinaemia. Overactivity of nuclear factor κB (NF-κB) is linked with inflammatory, cancerous and autoimmune diseases. The first case is described of an unusual life-threatening RDD of the trachea with no lymphadenopathy at risk of suffocation in a 39-year-old Chinese woman. A diagnosis of RDD was made following CT scans, thoracotomy and histological examination. Gel shift assay revealed an essential role for NF-κB overactivity in RDD. The patient remains well with no evidence of progression without treatment. Histological confirmation should be sought in all cases as the clinical manifestation of RDD is similar to asthma or lung carcinoma.
authors.affiliation: Department of Respiratory Medicine, The First Affiliated Hospital, Nanjing Medical University, Nanjing, Jiangsu 210029, China. lfzhou@njmu.edu.cn
authors.forename: Lin-Fu
authors.initials: LF
authors.lastname: Zhou
authors.forename: Liang
authors.initials: L
authors.lastname: Chen
authors.forename: Quan
authors.initials: Q
authors.lastname: Zhu
authors.forename: Cong
authors.initials: C
authors.lastname: Wang
authors.forename: Hai
authors.initials: H
authors.lastname: Xu
authors.forename: Xue-Fan
authors.initials: XF
authors.lastname: Cui
authors.forename: Lei-Fu
authors.initials: LF
authors.lastname: Jiang
authors.forename: Shao-Heng
authors.initials: SH
authors.lastname: He
authors.forename: Mao
authors.initials: M
authors.lastname: Huang
authors.forename: Kai-Sheng
authors.initials: KS
authors.lastname: Yin
mesh_terms: D000328:Adult; D000402:Airway Obstruction / Q000209:etiology; D024202:Electrophoretic Mobility Shift Assay; D005260:Female; D015618:Histiocytosis, Sinus / Q000150:complications / Q000175:diagnosis* / Q000378:metabolism; D006801:Humans; D016328:NF-kappa B / Q000502:physiology*; D014057:Tomography, X-Ray Computed; D014133:Tracheal Diseases / Q000150:complications / Q000175:diagnosis* / Q000378:metabolism
chemical_list: D016328:NF-kappa B