Primary effusion lymphoma (PEL) is a rare condition, which accounts for approximately 4% of all human immunodeficiency virus (HIV)-associated non-Hodgkin lymphomas. PEL has a predilection for body cavities and occurs in the pleural space, pericardium, and peritoneum. Without treatment, the median survival is approximately 2-3 months, and with chemotherapy, the median survival is approximately 6 months. We describe the case of a 47-year-old male with HIV and Kaposi's sarcoma who presented with complaints of abdominal pain and distension and was subsequently diagnosed with PEL. Despite limited clinical data being available, chemotherapy with dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin (EPOCH) has proven to increase survival rates in patients with this condition.
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