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A Rare Presentation of Rosai Dorfman Disease FREE TO VIEW

Lakshmi Varadarajalu, MD; Sunder Thirugnanasambandan, MS; Paul Jude Thangaraj, MS; Ashok Parameswaran, MD; Meghena Mathew*, MBBS
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Apollo Hospitals, Chennai, India

Chest. 2012;142(4_MeetingAbstracts):998A. doi:10.1378/chest.1389638
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SESSION TYPE: Miscellaneous Global Case Report Posters

PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM

INTRODUCTION: Rosai- Dorfman disease is a rare histiocytic disorder of unknown etiology commonly involving the cervical lymph nodes first described in 1969 , since then more than 400 cases have been reported in the Rosai Dorfman disease registry.1 Extranodal involvement have been reported in about 20-40 % of cases which includes skin, soft tissue, upper respiratory tract, bones , eye, urogenital tract, breast, gastrointestinal tract and lung. Isolated intrathoracic manifestation has been reported to be as high as 43% from a retrospective analysis .2 We report a case of extranodal involvement of the lung in a young male who presented with massive hemoptysis requiring a pneumonectomy.

CASE PRESENTATION: : A 28 year old male presented to the emergency department with massive hemoptysis of one day duration .On examination he was hemodynamically stable. There was decreased breath sounds over base of left lung . Laboratory investigations were non diagnostic. CT chest showed left lower lobe subpleural nodular opacities. He underwent fibreoptic bronchoscopy which showed active bleeding from left lower lobe bronchus. Bronchial artery angiogram revealed anomalous blood supply to left lower lobe from right bronchial artery and abnormal cork screw vessels to apical segment of left lower lobe from left superior intercostal arteries, which were embolised . Despite embolization, he had recurrent massive hemoptysis. Thoracic surgery consult was obtained. He was taken up for emergency left lower lobectomy. Intraoperatively , there were numerous abnormal vessels over surface of entire left lung. The hilum was found to be inflamed with numerous abnormal vessels over left main bronchus which was densely adherent to main pulmonary artery. Inflammatory adhesions with nodes in the fissure precluded a safe lower lobectomy, thereby necessitating a left pneumonectomy. Microscopy of the specimen revealed lung tissue composed of plasma cells, neutrophils , histiocytes and large cells with vesicular nuclei and abundant cytoplasm exhibiting emperipolesis consistent with diagnosis of Rosai Dorfman Disease .With no further symptoms he is under clinical observation.

DISCUSSION: : Rosai Dorfman Disease is a histiocytosis involving lymph nodes and characterized by sinus infiltrated with histiocytes, lymphocytes and plasma cells. The exact mechanism of etiology remains unknown. A cytokine-mediated migration of monocytes may be involved in histiocytic accumulation and activation. The trigger for this cytokine activation has been hypothesized to be secondary to infections, auto-immune disorders and hematological malignancies , however there is no strong evidence for the same .Viruses like Herpesvirus 6 and Epstein-Barr virus have been implicated as potential causative agents . Currently, it is believed that defective Fas/FasL signaling which leads to altered apoptosis may be a significant mechanism causing uncontrolled histiocytic proliferation.3 The disease manifests commonly in the first and second decade of life. Histologically the presence of emperipolesis (engulfment of lymphocytes and erythrocytes by histiocytes) is considered diagnostic. Immunohistochemistry demonstrates positivity for S-100, CD68, CD163, alpha-1 antichymotrypsin, alpha-1 antitrypsin, fascin and HAM-56 and CD1a negativity. The treatment for RDD is not well defined. Management options are few and vary from observation to surgical debulking. Systemic corticosteroids, chemotherapy and radiotherapy have all been attempted in patients with severe symptoms.

CONCLUSIONS: RDD is a rare disorder which usually involves only lymph nodes and is generally self-limiting. However , in rare instances ,can also involve organs such as the lungs and produce life threatening hemoptysis.

1) Henter JI, Tondini C, Pritchard J: Histiocyte disorders. Crit Rev Oncol Hematol 2004;50(2):157-174.

2) Cartin- Ceba R , Golbin JM, Yi ES, Prakash UB, Vassallo R. Intrathoracic manifestations of Rosai-Dorfman disease. Respir Med. 2010;Sep;104(9):1344-1349

3) McClain KL, Natkunam Y, Swerdlow H: Atypical cellular disorders. Hematology Am Soc Hematol Educ Program . 2004;1:283-96

DISCLOSURE: The following authors have nothing to disclose: Lakshmi Varadarajalu, Sunder Thirugnanasambandan, Paul Jude Thangaraj, Ashok Parameswaran, Meghena Mathew

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Apollo Hospitals, Chennai, India




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