Unusual life-threatening Rosai-Dorfman disease of the trachea: role of NF-κB.
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.
Zhou
Lin-Fu
LF
Department of Respiratory Medicine, The First Affiliated Hospital, Nanjing Medical University, Nanjing, Jiangsu 210029, China. lfzhou@njmu.edu.cn
Chen
Liang
L
Zhu
Quan
Q
Wang
Cong
C
Xu
Hai
H
Cui
Xue-Fan
XF
Jiang
Lei-Fu
LF
He
Shao-Heng
SH
Huang
Mao
M
Yin
Kai-Sheng
KS
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
D016328:NF-kappa B