CASE DESCRIPTION
A 41-year-old HIV-positive male presented to the emergency department
with fever, malaise, and shortness of breath. Splenomegaly was noted on
physical exam but no lymphadenopathy or cavitary effusions. His viral
load was markedly elevated with CD4 count of 16 cells/µL, and he was
pancytopenic. Clinical suspicion was high for infection, acute leukemia,
or hemophagocytic lymphohistiocytosis. Bone marrow biopsy showed sheets
of large atypical mononuclear cells with open chromatin, distinct
nucleoli, and ample basophilic cytoplasm (Figures 1A & B). Tumor cells
were positive for CD138 (Figure 1C) and CD30 (Figure 1D) while negative
for CD45, CD3, CD20, PAX5, OCT2, and CD79a. Differential diagnoses
included plasmablastic lymphoma, plasma cell myeloma with plasmablastic
features, and primary effusion lymphoma (PEL). Both EBV by in situ
hybridization (Figure 1E) and HHV-8 (Figure 1F) were positive in tumor
cells; hence, the diagnosis of extracavitary PEL was rendered.
Chemotherapy with EPOCH was initiated; nevertheless, the patient died of
disease 19 days after diagnosis.
PEL is a distinct clinicopathologic entity of AIDS-related lymphomas.
Extracavitary PEL is a rare clinical variant of PEL that presents with
solid tumor lesions in absence of malignant serous
effusions.1,2 This case highlights the importance of
recognizing PEL outside of cavitary lesions.