Unusual life-threatening Rosai-Dorfman disease of the trachea: role of NF-kappa B

Author:

Zhou, Lin-Fu  Chen, Liang  Zhu, Quan  Wang, Cong  Xu, Hai  Cui, Xue-Fan  Jiang, Lei-Fu  He, Shao-Heng  Huang, Mao  Yin, Kai-Sheng  

Journal:

THORAX

Issue Date:

2010

Abstract(summary):

RosaieDorfman 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 kappa B (NF-kappa B) 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-kappa B 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.

Page:

927---929