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: 10 October 2022 (update in progress)

Copyright: 2001-2022, PathologyOutlines.com, Inc.

PubMed Search: Primary effusion lymphoma

Nikhil Sangle, M.D.
Page views in 2021: 3,861
Page views in 2022 to date: 4,477
Cite this page: Sangle N. Primary effusion lymphoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lymphomaeffusion.html. Accessed December 9th, 2022.
Definition / general
  • Rare type of diffuse large B cell lymphoma with lymphomatous effusions in pleural, pericardial and abdominal cavities but no tumor mass
Terminology
  • 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

Contributed by Mario L. Marques-Piubelli, M.D. and Roberto N. Miranda, M.D. (Case #519)
Extracavitary presentation of PEL

Extracavitary presentation of PEL

EBER

EBER

LANA-1

LANA-1

LANA-1 in an extracavitary presentation

LANA-1 in an extracavitary presentation



Images hosted on other servers:

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

Cytology images

Contributed by Mario L. Marques-Piubelli, M.D. and Roberto N. Miranda, M.D. (Case #519)
Diff-Quik

Diff-Quik

Wright-Giemsa

Wright-Giemsa

PEL Thinprep

PEL Thinprep

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
Board review style question #1
Which of the following pair of viruses are associated with primary effusion lymphoma (PEL)?

  1. EBV and HCV
  2. EBV and HHV-8
  3. HHV-8 and COVID-19
  4. HHV-8 and HPV
Board review style answer #1
B. EBV and HHV-8

Comment Here

Reference: Primary effusion lymphoma
Board review style question #2
Which is the latency pattern of the Epstein-Barr virus found associated with primary effusion lymphoma (PEL)?

  1. Latency Pattern 1: EBER(+), EBNA-1(+), LMP-1(-)
  2. Latency Pattern 1: EBER(+), EBNA-1(+), LMP-1(+)
  3. Latency Pattern 3: EBER(+), EBNA-2(-), LMP-1(+)
  4. Latency Pattern 2: EBER(+), EBNA-2(-), LMP-1(+)
Board review style answer #2
A. Latency Pattern 1: EBER(+), EBNA-1(+), LMP-1(-)

Comment Here

Reference: Primary effusion lymphoma
Back to top
Image 01 Image 02