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

Abstract

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.

Intrathoracic Rosai-Dorfman Disease with Spontaneous Remission: A Clinical Report and a Review of the Literature

“…Those are founded with accounting for 40% of RDD. On the other hand, intrathoracic manifestations of RDD are rare and represent only 2% of the cases with RDD (Foucar et al 1990), and only 9 cases of RDD with localized intrathoracic lesions have been reported (Table 1) (Ohori et al 2003;Shi et al 2009;Ali and Mackay 2009;Hida et al 2009;Zhou et al 2010;Roberts and Attanoos 2010;Cartin-Ceba et al 2010;Kaseda et al 2011). The discriminative pathological findings of the present patient were diffuse bronchial proliferation of histiocytes with round or oval shaped nuclei and clear and weakly acidophilic cytoplasm (McClain et al 2004).…”

“…It was reported that around 40% of RDD patients have lymph node disorders and extranodal involvement, but intrathoracic manifestations of RDD was reported in only 2% of patients with RDD (Foucar et al 1990). Furthermore, there have been only 9 cases of RDD with localized intrathoracic lesions (Ohori et al 2003;Shi et al 2009;Ali and Mackay 2009;Hida et al 2009;Zhou et al 2010;Roberts and Attanoos 2010;Cartin-Ceba et al 2010;Kaseda et al 2011). No standard treatment for RDD has yet been established, but a good prognosis has been reported (Foucar et al 1990), and another report (Gotou et al 2008) noted that 20% of the cases showed spontaneous remission.…”

Regulation of Th1/Th2 balance through OX40/OX40L signalling by glycyrrhizic acid in a murine model of asthma

“…GA also has protective role on β 2 adrenergic receptor (β 2 AR) agonist‐stimulated receptor internalization and cell apoptosis . The combination of GA and β 2 AR led to a coordinate anti‐inflammatory function through the suppression of NF‐κB activation . GA also exhibited a glucocorticoid‐like inhibitory effect on inflammation without affecting the serum cortisol level .…”

Tracheobronchial Involvement of Rosai–Dorfman Disease

“…Infiltration of the tracheobronchial tree may be responsible for an obstructive pattern on pulmonary function test. 22 On fiberoptic bronchoscopy, the size of tracheobronchial lesions ranged from granular infiltration to a 40 mm mass. Of note, subglottic stenosis can also be secondary to extrinsic compression by massive lymphadenopathy.…”