Primary effusion lymphoma involving three body cavities

Abstract

Primary effusion lymphoma (PEL) is a human herpes virus-8 (HHV8)-associated large-cell non-Hodgkin lymphoma localized in body cavities and presenting as pleural, peritoneal, or pericardial lymphomatous effusions. It typically affects immunocompromised patients and usually involves only one body site. We describe herein a case of PEL affecting three body cavity sites in an immunocompetent patient. A 69-year-old HIV-negative man presented with upper gastrointestinal bleeding and ascites. An examination of the fluid by cytology showed large atypical lymphocytes with abundant basophilic cytoplasm, either central or eccentric nuclei having irregular outlines, and multiple prominent nucleoli. The neoplastic cells showed positive staining for CD45, CD3, HHV8 latent nuclear antigen (LNA), and Epstein-Barr virus-encoded RNA. A diagnosis of PEL was rendered. Despite chemotherapy and valganciclovir, the disease progressed to involve the pleural and pericardial cavities and the patient died 5 months following the initial diagnosis. Although PEL is a B-cell lymphoma, it is usually of null phenotype by immunohistochemistry, and can rarely aberrantly express T-cell markers, as seen in the current case. The key to the diagnosis of PEL rests on identifying HHV8 in the neoplastic cells. Therefore, restricting the term of PEL only to those cases that are HHV8 positive is important in order to differentiate PEL from other lymphomas that can present as serous effusions and that carry, in general, a more favorable prognosis than PEL