SESSION TITLE: Miscellaneous Cases II
SESSION TYPE: Affiliate Case Report Slide
PRESENTED ON: Sunday, October 27, 2013 at 01:15 PM - 02:45 PM
INTRODUCTION: Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is mostly reported in Afro-Carribean population during the first or second decade of life. The definite etiology of this disease is unknown.
CASE PRESENTATION: A 33 year old African American woman was referred to pulmonary clinic for evaluation of dry cough and dyspnea on exertion for 3 years. Three years before, she presented to ED with cough and right sided pleuritic chest pain. CT chest performed in the ED revealed a soft tissue density in the right inferior hilum suggestive of post obstructive pneumonia or granulomatous process (Image 1a). Patient did not establish with a PCP until recently with the physician who referred her to us for a persistently abnormal CT chest which revealed increasing soft tissue density in the right inferior hilum with compression of the lower lobe bronchus suspicious for granulomatous disease or malignancy (Image 1b). Physical examination was unremarkable. Surgical biopsy revealed diffuse histiocytic and plasmacytic infiltration of the lung parenchyma with "emperipolesis" of plasma cells within the histiocytes (Image 2). The histiocytes were strongly positive for S100 and CD68 confirming RDD of the lung. Two months after diagnosis, the patient remains asymptomatic.
DISCUSSION: RDD is characterized by benign proliferation of histiocytes within lymph node sinuses and lymphatics in extra nodal sites. The most common presentation is bilateral massive painless cervical lymphadenopathy accompanied by fever and weight loss. Involvement of the larynx, lung, and pleura is rare and comprises 3% of extranodal cases. Cough and dyspnea are the most common presenting symptoms of RDD of the lung. Mediastinal lymphadenopathy is the most common intrathoracic manifestation followed by airway disease, pleural effusion, cystic and interstitial lung disease. The clinical course can be unpredictable with intermittent symptoms. The definitive treatment for RDD is yet to be identified. Patients can be observed if RDD lesions are not massive. Steroids may be initiated in case of fever > 38 C without documented infection or sudden lymph node enlargement. Surgical debulking or radiotherapy can be considered in life threatening situations due to massive lymphadenopathy. Various chemotherapeutic agents have been used without much success. Prognosis is variable.
CONCLUSIONS: RDD is metabolically active and shows increased uptake on the PET scan raising the suspicion for malignancy. RDD of the lung needs to be differentiated from carcinoma, melanoma, lymphoma, Langerhans cell histiocytosis, sarcoidosis, histoplasmasis, and tuberculosis. Treatment may not be necessary as the disease is often self-limiting.
Reference #1: Cartin-Ceba R, Golbin JM, Yi ES, Prakash UB, Vassallo R. Intrathoracic manifestations of Rosai-Dorfman disease. Respir Med. 2010;104(9):1344-9.
Reference #2: Ali A, Mackay D. Rosai-Dorfman disease of the lung. Thorax. 2009;64(10):908-9.
DISCLOSURE: The following authors have nothing to disclose: Vijaya Sivalingam Ramalingam, Tilottama Majumdar, Arjun Rao
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