Lymphoma & related disorders

Mature B cell neoplasms

Large B cell lymphomas-special subtypes

Primary effusion lymphoma

Last author update: 1 March 2011
Last staff update: 14 June 2022

Copyright: 2001-2022,, Inc.

PubMed Search: primary effusion lymphoma

Nikhil Sangle, M.D.
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Cite this page: Sangle N. Primary effusion lymphoma. website. Accessed July 6th, 2022.
Definition / general
  • Rare type of diffuse large B cell lymphoma with lymphomatous effusions in pleural, pericardial and abdominal cavities but no tumor mass
  • Also called body cavity lymphoma
Clinical features
  • Only one body cavity typically involved
  • Strongly associated with HHV8 and advanced HIV; usually EBV+
  • Also occurs in nonimmunosuppressed patients, often elderly
  • Rarely, extracavitary tumors with features of PEL are seen in GI tract, skin, lung, CNS and lymph nodes
  • Survival usually only months
  • Solid variant: very rare; affects GI tract, skin, lung, cerebrum; rarely nodal (Hum Pathol 2002;33:846); may represent a heterogeneous group of disorders
Case reports
Microscopic (histologic) description
  • Immunoblastic cells with abundant basophilic cytoplasm, round nucleus, single prominent nucleoli, perinuclear halo, variable nuclear pleomorphism with binucleated cells
  • Occasional large Reed-Sternberg like and anaplastic cells
  • Rarely is solid with plasmablastic features
  • Solid variant: large pleomorphic cells; nodal case resembled anaplastic large cell lymphoma
Microscopic (histologic) images

Images hosted on other servers:

70 year old man with HHV8+, EBV+ pleural effusions

Positive stains
Negative stains
Molecular / cytogenetics description
  • Often required for diagnosis since negative for B cell stains
  • Clonal rearrangements and somatic mutations of Ig heavy chain
  • Clonal Epstein Barr virus and HHV8 genetic sequences
  • Also gain of sequences in chromosomes 12 and X (Arch Pathol Lab Med 2000;124:824)
Differential diagnosis
Additional references
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