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Sarcoidosis-Lymphoma Syndrome: A Diagnostic Dilemma FREE TO VIEW

Assad Oskuei, MD; Tawnya Hansen, MD; Victor Hoffstein, MD
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St. Michael's Hospital, University of Toronto, Toronto, ON, Canada

Chest. 2014;146(4_MeetingAbstracts):677A. doi:10.1378/chest.1993936
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SESSION TITLE: Cancer Student/Resident Cases

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 28, 2014 at 04:30 PM - 05:30 PM

INTRODUCTION: Sarcoidosis-Lymphoma syndrome is a rare entity that provides a diagnostic challenge for clinicians. Peripheral lymph node (LN) biopsy, when possible, has a high diagnostic yield. Here we present a case of sarcoidosis and lymphoma with negative mediastinal and peripheral LN biopsies.

CASE PRESENTATION: A previously healthy 43-year old female presented with a productive cough and fever that had failed multiple outpatient oral antibiotic therapy. Physical examination revealed hypoxemia on exertion, diffuse bilateral crackles and a palpable left axillary LN. Her labs were unremarkable. Chest CT showed bilateral areas of consolidation, mediastinal and left axillary lymphadenopathy, as well as hepatosplenomegaly. Bronchoscopy and bronchoalveolar lavage were performed; cytology, TB smear, bacterial and fungal cultures were non-diagnostic. Axillary LN biopsy was negative. Subsequently, the patient was treated for presumed cryptogenic organizing pneumonia with prednisone and had clinical improvement. Once prednisone was discontinued, she developed constitutional symptoms, Bell’s palsy, elevated ACE levels and hypercalcemia. Mediastinal LN biopsy was negative but the video-thoracoscopic lung biopsy revealed non-necrotizing granulomas. Sarcoidosis was diagnosed. Steroids were reinstituted. However, over the next year, she continued to have recurrent B-symptoms, unexplained peripheral lymphadenopathy and rapidly progressive splenomegaly. Multiple bone marrow and submandibular LN biopsies were non-diagnostic. Eventually, a therapeutic and diagnostic splenectomy was performed which showed terminally differentiated B-cell lymphoma, specifically marginal zone lymphoma. Splenic parenchyma contained multifocal non-necrotizing granulomata resembling those noted in the previous lung wedge resection. She became asymptotic after the splenectomy.

DISCUSSION: Lymphoma tends to rarely surface in chronic active sarcoidosis and this coexistence is thought to be due to immunological abnormalities that occur in the latter disease. The spleen is involved in approximately 7% of sarcoidosis and 50% of lymphoma patients. Splenic marginal zone lymphoma accounts for about 1% of B-Cell lymphomas. To our knowledge, this is the second reported case of sarcoidosis and splenic marginal zone lymphoma in the literature.

CONCLUSIONS: In patients with sarcoidosis, new lymphadenopathy and splenomegaly needs further investigation for coexistence of lymphoma. The spleen may be the only site revealing the diagnosis.

Reference #1: Brincker H, Wilbek E. The incidence of malignant tumours in patients with respiratory sarcoidosis. Br J Cancer 1974; 29: 247-251.

Reference #2: Yanardag H, Caner M, Papila I, Uygun S, Demirci S, Karayel T. Diagnostic value of peripheral lymph node biopsy in sarcoidosis: A report of 67 cases. Can Respir J. 2007. 14(4): 209-211.

Reference #3: Papanikolaou I. C, Sharma O.P, The relationship between sarcoidosis and lymphoma. Eur Respir J 2010; 36: 1207-1219

DISCLOSURE: The following authors have nothing to disclose: Assad Oskuei, Tawnya Hansen, Victor Hoffstein

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