Primary effusion lymphoma

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

Primary effusion lymphoma (PEL) is a B-cell lymphoma.[1]

Causes[edit]

It is caused by Kaposi's sarcoma-associated herpesvirus (KSHV), also known as human herpesvirus 8 (HHV-8).[2][3]

In most cases, the lymphoma cells are also infected with Epstein Barr virus (EBV).[4]

PEL most commonly arises in patients with underlying immunodeficiency, such as AIDS.[5][6]

The condition can exist in the absence of HHV-8 and HIV, though this is rare.[7]

Presentation[edit]

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".

A case has been described that was positive for CD38, CD71 and CD30.[8] Positive also for CD45 (LCA), CD138, MUM-1 but negative for CD20, CD79a and PAX-5.

History[edit]

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

Prognosis[edit]

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[edit]

References[edit]

  1. ^ "primary effusion lymphoma" at Dorland's Medical Dictionary
  2. ^ 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. doi:10.1056/NEJM199505043321802. PMID 7700311. 
  3. ^ 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. doi:10.1158/0008-5472.CAN-03-3835. PMID 15256448. 
  4. ^ 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. doi:10.1128/JVI.79.2.1244-1251.2005. PMC 538532. PMID 15613351. 
  5. ^ Boshoff C, Weiss R (May 2002). "AIDS-related malignancies". Nat. Rev. Cancer 2 (5): 373–82. doi:10.1038/nrc797. PMID 12044013. 
  6. ^ 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. doi:10.1038/ncponc0253. PMID 16130937. 
  7. ^ Youngster I, Vaisben E, Cohen H, Nassar F (January 2006). "An unusual cause of pleural effusion". Age Ageing 35 (1): 94–6. doi:10.1093/ageing/afj009. PMID 16364944. 
  8. ^ Chen YB, Rahemtullah A, Hochberg E (May 2007). "Primary effusion lymphoma". Oncologist 12 (5): 569–76. doi:10.1634/theoncologist.12-5-569. PMID 17522245. 
  9. ^ 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. doi:10.1182/blood-2006-06-028092. PMC 1801055. PMID 17082322.