Primary effusion lymphoma (PEL) or body cavity lymphoma is a rare type of extra nodal lymphoma of B-cell origin that presents as lymphomatous effusion(s) without any nodal enlargement or tumor masses. It belongs to the group of AIDS related non-Hodgkin’s lymphomas. First described in 1996 in HIV infected individuals who were coinfected with Kaposi’s sarcoma-associated herpesvirus (KSHV) or HHV-8 virus, it was included as a separate entity in WHO classification of tumors of hematopoietic and lymphoid tissue in the year 2001. The definition included association with HHV-8 virus as a mandatory diagnostic criterion. However, cases were later reported where PEL-like disease process was diagnosed in HHV-8 negative patients. This was eventually recognized as a rare but distinct entity termed as “HHV-8-unrelated PEL-like lymphoma”. Herein, we are reporting a case of an elderly patient who presented with a large pleuropericardial effusion and was eventually diagnosed with this entity. Till date, only around 50 cases of HHV-8-unrelated PEL-like lymphoma have been reported and our case being EBV, HIV, and Hepatitis C negative makes it very unique and rare occurrence. We are also presenting a review of relevant literature focused mainly on comparing outcomes in patients treated with and without chemotherapy. 1. Case Presentation A 76-year-old ex-smoker male with past medical history of hypertension and atrial fibrillation presented with exertional dyspnea and a recent weight loss of 15？lbs. He denied substance abuse. Review of systems and EKG were negative. On physical examination, breath sounds at bilateral lung bases were decreased. The superficial lymph nodes, liver, and spleen were not palpable on physical examination. There was no lower extremity edema. Hemogram revealed a WBC count of 10,700？cells/mm3 with 80% granulocytes, hemoglobin of 12.3？g/dL, and platelet count of 303000/microl. Cardiac enzymes were normal. Thyroid function tests were normal. Chemistry showed mild elevation of alkaline phosphatase and GGT. Lactate dehydrogenase was 158？U/L and ESR was 23？mm/hr. Chest radiography showed enlarged cardiac silhouette and small bilateral pleural effusions. Echocardiography showed a large pericardial effusion. He underwent pericardiocentesis with removal of 800？mL of hemorrhagic fluid. Symptoms improved and patient was discharged home to follow up as an outpatient. Pericardial fluid analysis revealed 12,300 leukocytes with 90% monocytes, 246,000 erythrocytes, a protein of 4.7？g/dL, and LDH of 6,000？IU/L. The fluid was cellular and cytology showed atypical
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