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Extranodal Marginal Zone Lymphoma of the Pleura Presenting as Unilateral Pleural Effusion: A Case Report FREE TO VIEW

Gabriel Lockhart, MD; Manu Kaushal, MD; Asma Azimi, MD; Patrick Nana-sinkam, MD
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The Ohio State Wexner Medical Center, Columbus, OH

Chest. 2014;146(4_MeetingAbstracts):657A. doi:10.1378/chest.1991967
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SESSION TITLE: Cancer Student/Resident Case Report Posters II

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Extranodal Marginal Zone Lymphoma (EMZL) is a well-described B-cell lymphoma of the mucosa associated lymphoid tissue. Disease presents most commonly in the gastrointestinal tract; however we report a rare case of EMZL with primary pleural involvement.

CASE PRESENTATION: A 50 year-old female presented with 3 months of progressive right upper quadrant abdominal pain and diarrhea following a laparoscopic cholecystectomy. Initial evaluation included a computed tomography of the abdomen which was unrevealing with the exception of an incidental large right-sided pleural effusion. Diagnostic thoracentesis revealed lymphocytic predominant exudative fluid with negative bacterial and fungal cultures, and markedly reactive lymphocytosis and mesothelial cells on cytology. Gastroenterology workup at that time was nondiagnostic. The patient was then discharged with recommendation to follow up at an outpatient clinic. Prior to follow up, the patient was re-admitted to the hospital 4 weeks later with complaints of worsening dyspnea and cough with clear sputum. Her review of systems was otherwise positive for low-grade fevers, malaise, and weight loss. A chest radiograph revealed worsening right sided pleural effusion. A repeat thoracentesis had similar results as prior but additionally was positive for EBV by PCR and a population of CD3 and CD20 positive T cells with loss of CD26 . She ultimately underwent video-assisted thoracic surgery with pleural biopsy. Pathology from the pleura showed lymphocytic pleuritis with atypical features. Immunohistochemistry of the pleural sample demonstrated a focal expansion of marginal zones. IgH rearrangements demonstrated a monoclonal population of B cells. The results were interpreted as consistent with extra-nodal marginal zone lymphoma. Peripheral blood flow cytometry and bone marrow analysis were later performed and were negative for any abnormality.

DISCUSSION: The more typical forms of primary pleural lymphoma present as primary effusion lymphoma or pyothorax-associated lymphoma. EMZL is a rare form of lymphoma, described in eight case reports1,2. EMZL comprises approximately 5% of all non-Hodgkins lymphomas, a low-grade, usually indolent B cell lymphoma. Immunophenotyping is usually positive for B cell markers CD19, CD20, and CD22, and negative for CD5, CD10, and CD23. While it is not clear what disease associations predispose individuals to the development of EMZL, it has been postulated that it results from chronic immune stimulation (infectious, inflammatory).

CONCLUSIONS: Although rare, primary pleural EMZL cases have been reported. We describe this case to raise awareness of primary pleural EMZL/MALT as a cause of recurrent pulmonary effusion.

Reference #1: Mitchell et al. Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue With Initial Presentation in the Pleura. CHEST 2006; 129:791-794.

Reference #2: Xiaohu et al. Primary pleural lymphoma: a rare case and a synopsis of the literature J Thorac Dis 2013;5(4):E121-E123.

DISCLOSURE: The following authors have nothing to disclose: Gabriel Lockhart, Manu Kaushal, Asma Azimi, Patrick Nana-sinkam

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