An unusual presentation of body cavity lymphoma

Abstract

6699 Background: BCL (Body Cavity Lymphoma) is an uncommon primary NHL (Non-Hodgkin's lymphoms) that proliferates within serous body cavities - pleural, pericardial or peritoneal, resulting in recurrent effusions. It is common in young to middle-aged HIV (Human Immunodeficiency Virus) positive homosexual males. In the absence of HIV infection, it almost universally develops in the background of HHV-8/KSHV (Human Herpes Virus-8/ Kaposi's Sarcoma Herpes Virus) infection with or without EBV (Epstein Barr Virus). Rare BCLs have been reported in elderly populations, which are both HIV negative and HHV-8 negative. We report an unusual case of BCL, which is HHV-8 negative and EBV positive. Abstract: 75 yrs old Immigrant Ashkenazi Jew from Russia was admitted with shortness of breath for 1 month and leg edema. No B-symptoms. His Karnofsky performance status was 80. He is heterosexual, monogamous, non-smoker and denied alcohol or intravenous drug abuse. Clinical examination revealed a right-sided pleural effusion only. Complete Blood Count showed mild normocytic normochromic anemia with hemoglobin -11.5 gm/dl. Total white count, differential, renal and liver function tests were normal. Serum LDH 200 U/L, serum β2 Microglobulin 2.8. Pleural fluid was frankly hemorrhagic with 80,000 RBC/cu mm, 16,800 WBC/ cu mm with 65% lymphocytes and 26% monocytes. Pleural fluid LDH 4500 U/L and cytology revealed Large cell lymphoma. CT scan of chest, abdomen and pelvis failed to demonstrate any lymphadenopathy or organomegaly. There was no pericardial effusion or ascites. Pleural fluid flow-cytometry demonstrated monoclonal lambda B-cell population lacking CD5 or CD10. The cells were positive for CD19, CD20, CD22 and CD45 and negative for CD2, CD3, CD4, CD5, CD7, CD9, CD10, CD11c and CD13/33. Bone marrow aspiration biopsy and flow-cytometry were negative for disease. Serum PCR for HHV-8, Human T Cell Leukemia Virus 1 and 2, Hepatitis C Virus negative, serum EBV IgG positive. CD4 count was 400 and CD8 was 300 with a ratio of 1.3. A diagnosis of BCL was made. He underwent therapeutic thoracentesis with relief of respiratory symptoms.Standard CHOP-like chemotherapy has been planned for this patient. No significant financial relationships to disclose.