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Primary effusion lymphoma

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Title: Primary effusion lymphoma  
Author: World Heritage Encyclopedia
Language: English
Subject: International Classification of Diseases for Oncology, BCP-1 cells, VG-1, Pel, Kaposi's sarcoma-associated herpesvirus
Collection: Epstein–barr Virus-Associated Diseases, Infectious Causes of Cancer, Lymphoma
Publisher: World Heritage Encyclopedia

Primary effusion lymphoma

Primary effusion lymphoma
Classification and external resources
Specialty Hematology and oncology
ICD-O M9678/3
DiseasesDB 33904
MeSH D054685

Primary effusion lymphoma (PEL) is a B-cell lymphoma, presenting with a malignant effusion without a tumor mass.[1][2]:879-881


  • Causes 1
  • Presentation 2
  • History 3
  • Prognosis 4
  • See also 5
  • References 6


PEL most commonly arises in patients with underlying immunodeficiency, such as AIDS.[3][4] PEL is caused by Kaposi's sarcoma-associated herpesvirus (KSHV), also known as human herpesvirus 8 (HHV-8).[5][6] In most cases, the lymphoma cells are also infected with Epstein Barr virus (EBV).[7]

The condition can exist in the absence of HHV-8 and HIV, though this is rare.[8]


PEL is unusual in that the majority of cases arise in body cavities, such as the pleural space or the pericardium; another name for PEL is "body cavity lymphoma".

The immunophenotype : CD45+ (95%), CD20-, CD79a-, PAX5-, CD30+, CD38+, CD138+ and EMA+.[2]:879-881 Staining the nucleus for HHV-8 LANA may be helpful.[2]:879-881


It was recognized as a unique type of lymphoma only after the discovery of KSHV in 1994.


It is generally resistant to cancer chemotherapy drugs that are active against other lymphomas, and carries a poor prognosis.[9]

Sirolimus has been proposed as a treatment option.[10]

See also


  1. ^ Swerdlow, Steven H.; International Agency for Research on Cancer; World Health Organization (2008). WHO classification of tumours of haematopoietic and lymphoid tissues. World Health Organization classification of tumours 2 (4th ed.). International Agency for Research on Cancer.  
  2. ^ a b c Jaffe, ES; Harris NL, Vardiman JW, Campo E, Arber, DA. (2011). Hematopathology (1st ed.). Elsevier Saunders.  
  3. ^ Boshoff C, Weiss R (May 2002). "AIDS-related malignancies". Nat. Rev. Cancer 2 (5): 373–82.  
  4. ^ Yarchoan R, Tosato G, Little RF (August 2005). "Therapy insight: AIDS-related malignancies--the influence of antiviral therapy on pathogenesis and management". Nat Clin Pract Oncol 2 (8): 406–15; quiz 423.  
  5. ^ Cesarman E, Chang Y, Moore PS, Said JW, Knowles DM (May 1995). "Kaposi's sarcoma-associated herpesvirus-like DNA sequences in AIDS-related body-cavity-based lymphomas". N. Engl. J. Med. 332 (18): 1186–91.  
  6. ^ Staudt MR, Kanan Y, Jeong JH, Papin JF, Hines-Boykin R, Dittmer DP (July 2004). "The tumor microenvironment controls primary effusion lymphoma growth in vivo". Cancer Res. 64 (14): 4790–9.  
  7. ^ Fan W, Bubman D, Chadburn A, Harrington WJ, Cesarman E, Knowles DM (January 2005). "Distinct subsets of primary effusion lymphoma can be identified based on their cellular gene expression profile and viral association". J. Virol. 79 (2): 1244–51.  
  8. ^ Youngster I, Vaisben E, Cohen H, Nassar F (January 2006). "An unusual cause of pleural effusion". Age Ageing 35 (1): 94–6.  
  9. ^ Chen YB, Rahemtullah A, Hochberg E (May 2007). "Primary effusion lymphoma". Oncologist 12 (5): 569–76.  
  10. ^ Sin SH, Roy D, Wang L, et al. (March 2007). "Rapamycin is efficacious against primary effusion lymphoma (PEL) cell lines in vivo by inhibiting autocrine signaling". Blood 109 (5): 2165–73.  
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