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Lymphoma - B cell neoplasms

Primary effusion lymphoma (PEL)


Reviewer: Nikhil Sangle, M.D., University of Utah and ARUP Laboratories (see Reviewers page)
Revised: 8 January 2012, last major update March 2011
Copyright: (c) 2001-2012, PathologyOutlines.com, Inc.

Definition
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● Rare type of diffuse large B cell lymphoma with lymphomatous effusions in pleural, pericardial and abdominal cavities but no tumor mass

Terminology
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● Also called body cavity lymphoma

Clinical features
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● Only one body cavity typically involved
● Strongly associated with HHV8 and advanced HIV; usually EBV+
● Also occurs in non immunosuppressed 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
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● 37 year old HIV+ man with coexisting small bowel mass (Am J Surg Pathol 2002;26:1363)
● 42 year old HIV+ man with pleural cavity and lingual tumor (Hum Pathol 2004;35:632)
● 68 year old HIV negative but HHV8+ patient (Arch Pathol Lab Med 2000;124:753)
● 70 year old man with HHV8+, EBV+ pleural effusions (J Med Case Reports 2011;5:60)
● 87 year old HIV- but HHV8+ man with T cell variant (Arch Pathol Lab Med 2001;125:1246)
● Two cases of HIV+, HHV8+ solid variant without primary effusion lymphoma (Hum Pathol 2002;33:846)

Micro description
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● 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

Micro images
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68 year old HIV negative but HHV8+ patient


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


87 year old HIV- but HHV8+ man with T cell variant

Cytology images
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Various images: #1     #2     #3

Positive stains
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● CD30, CD138, EMA, HHV8
● Also HLA-DR, CD45, HHV8 associated latent protein (LANA/ORF73), EBV EBER
● Variable CD3

Negative stains
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● CD5, CD19, CD20, CD22, CD23, CD43, CD79a, immunoglobulins, p53, bcl6

Molecular
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● Often required for diagnosis since negative for B cell stains
● Clonal rearrangements and somatic mutations of Ig heavy chain
● Clonal Epstein-Barr virus and HHV-8 genetic sequences
● Also gain of sequences in chromosomes 12 and X (Arch Pathol Lab Med 2000;124:824)

Molecular images
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PCR for HHV8 and PCR

   
T cell variant: PCR for T cell receptor; PCR for HHV8

Differential diagnosis
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Plasmablastic lymphoma: not an effusion, positive for B cell markers
Pyothorax associated lymphoma: different clinical history, positive for B cell markers, HHV8-
● Solid variant (HHV8+) resembles diffuse large B cell lymphoma with plasmablastic features (HHV8-)

Additional references
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Mod Pathol 2002;15:944

End of Lymphoma - B cell neoplasms > Primary effusion lymphoma (PEL)


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