An Uncommon Cause of Refractory Ascites in an Immunocompromised Patient

Abstract

Purpose: Primary Effusion Lymphoma (PEL) is a rare form of Non-Hodgkin's lymphoma that involves serous body cavities (pleural, pericardial, or peritoneal) with lymphomatous effusions in the absence of lymphadenopathy or organomegaly. PEL is associated with HIV, HHV-8, EBV(formally designated as Human Herpes Virus 4), Hepatitis B or Hepatitis C. PEL is one of the rarest of the AIDS-related lymphomas, accounting for less than 1-4 percent of cases. PEL often presents with rapidly progressive effusions and generally has a poor prognosis. By flow cytometry, the antigens expressed in PEL are CD38, CD71, HLA-DR, CD30, and CD45. B-cell markers are usually negative. Cytomorphologically, all cases show atypical to anaplastic morphology. Median survival after diagnosis without treatment is approximately 2 to 3 months. Even with aggressive chemotherapy, median survival is extended to only 6 months. Methods: We describe a case of a 28-year-old African American male patient with a past medical history of AIDS and an absolute CD 4 T lymphocyte count of 35 cells/ml and non-compliance with Highly Active Anti Retroviral Therapy (HAART) presenting with abdominal pain, fever and shortness of breath. Patient was found to have ascites and pleural effusion on admission. The analysis of the ascitic and pleural fluid showed the effusion was most likely malignant. An examination of the fluid by cytology showed large atypical lymphocytes with abundant basophilic cytoplasm and irregular nuclei containing multiple prominent nucleoli. Flow cytometry of the effusion turned out to be a B cell lymphoma. The neoplastic cells showed strong positivity for HLA DR, CD 20, CD 30, CD 38, CD 45, EBV RNA (on in situ hybridization), cytoplasmic lambda light chain and focal positivity for HHV 8 Latency Associated Nuclear Antigen (LANA). He had recurrent episodes of ascites, pleural and pericardial effusion despite repeated pleural and ascitic fluid drainage. The patient expired on day 42, before initiation of chemotherapy, because of hypoxic respiratory failure. Conclusion: It is essential for clinicians and pathologists to have a high index of suspicion of PEL in HIV positive patients who have effusions without solid tumors or lymphadenopathy. Basic immunohistochemistry is essential for definitive diagnosis.