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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