Background: Primary effusion lymphoma (PEL) is a lymphoid proliferation related to Kaposi sarcoma herpesvirus 8/human herpesvirus 8 (KSHV/HHV8) that affects mainly human immunodeficiency virus (HIV) infected individuals but can also occur in other immunodeficiency settings. It is characterized by lymphomatous effusions in different serous body cavities without the presence of a detectable tumor mass. The diagnosis is challenging and the clinical outcomes are poor. Aim: The aim of this paper is to report a rare case of PEL in a man who have sex with women (MSW) with HIV-1/2 infection, history of visceral Kaposi sarcoma (KS) and the development of a seronegative arthritis previous to the lymphoproliferative disease diagnosis. PEL presented with ascites, was treated with high-dose chemotherapy and autologous stem cell transplantation, with a good clinical outcome. Case Presentation: We describe a case of a 48-year-old HIV-1/2-infected patient from a high HHV8 seroprevalent country, hospitalized following a three-month history of increased abdominal volume and general constitutional symptoms. Laboratory data revealed normocytic normochromic anemia and a high level of lactate dehydrogenase. A diagnostic paracentesis was performed with cytology compatible with high-grade B-cell lymphoma. Peritoneal fluid cytology showed large lymphoid cells expressing leucocyte-common antigen CD45 without expression of the CD20 antigen (B-lymphocytes) and positivity for HHV8 by immunocytochemical staining, compatible with the diagnosis of PEL.
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