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 kB (NF-kB) 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-kB 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.Sinus histiocytosis with massive lymphadenopathy (SHML) is a syndrome first recognised by Rosai and Dorfman in 1969. 1 As a rare non-neoplastic histiocytic proliferative disorder, RosaieDorfman disease (RDD) is characterised by bilateral painless cervical lymphadenopathy as well as low fever, high erythrocyte sedimentation rate, leucocytosis and hypergammaglobulinaemia. Extranodal involvement including the skin, orbit and nasopharynx occurs in approximately one-third of patients.
2Overactivity of nuclear factor kB p50/p65) is linked with inflammatory, cancerous and autoimmune diseases. 3 We describe an uncommon case of life-threatening RDD of the trachea in association with NF-kB overactivity.