Primary Effusion Lymphoma
Primary Effusion Lymphoma
Updated: 08/19/2024
© Jun Wang, MD, PhD
General features
- Currently classified as KSHV/HHV8-associated B-cell lymphoid proliferations and lymphomas
- Also called body cavity large B cell lymphoma
- Associated with HHV8 and advanced HIV infection
- Commonly EBV+
- High grade
- Very aggressive
- Poor prognosis.
- Diagnosis based on clonal rearrangement or mutations of Ig heavy chains due to negative B-cell markers
Clinical presentations
- Effusion, commonly single body cavity
- Usually no identifiable tumor mass
Key pathogenesis
- Human herpes virus 8
- HIV
- EB virus
Key Laboratory findings
- High grade tumor cells in effusions, pleural, pericardial or peritoneal
Key morphological features of
tumor cells
Markers
- Positive: CD30, CD138, HHV8, EMA
- Negative: CD5, CD19, CD20, CD79a
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