Primary effusion lymphoma

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

Primary effusion lymphoma
SpecialtyHematology and oncology

Presentation

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

Causes

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] In the cases, the World Health Organization (2016) classifies the disease as one of the Epstein-Barr virus-associated lymphoproliferative diseases, i.e. EBV+ primary effusion lymphoma. EBV is found in the malignant HHV8+ B cells in ~70% of PEL patients. However, a role for this virus in the development of PEL is not supported since HHV8 appears to drive the development and progression of the disease.[8] PEL condition can exist in the absence of HHV-8 and HIV, though this is rare.[9]

Prognosis

It is generally resistant to cancer chemotherapy drugs that are active against other lymphomas, and carries a poor prognosis.[10]

Sirolimus has been proposed as a treatment option.[11]

History

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

See also

References
  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. ISBN 9789283224310.
  2. Jaffe, ES; Harris NL; Vardiman JW; Campo E; Arber, DA. (2011). Hematopathology (1st ed.). Elsevier Saunders. ISBN 9780721600406.
  3. Boshoff C, Weiss R (May 2002). "AIDS-related malignancies". Nat. Rev. Cancer. 2 (5): 373–82. doi:10.1038/nrc797. PMID 12044013.
  4. Yarchoan R, Tosato G, Little RF (August 2005). "Therapy insight: AIDS-related malignancies--the influence of antiviral therapy on pathogenesis and management" (PDF). Nat Clin Pract Oncol. 2 (8): 406–15, quiz 423. doi:10.1038/ncponc0253. PMID 16130937.
  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. doi:10.1056/NEJM199505043321802. PMID 7700311.
  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. doi:10.1158/0008-5472.CAN-03-3835. PMID 15256448.
  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. doi:10.1128/JVI.79.2.1244-1251.2005. PMC 538532. PMID 15613351.
  8. Rezk SA, Zhao X, Weiss LM (June 2018). "Epstein - Barr virus - associated lymphoid proliferations, a 2018 update". Human Pathology. doi:10.1016/j.humpath.2018.05.020. PMID 29885408.
  9. 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.
  10. 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.
  11. 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.
Classification
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