Primary effusion lymphoma

Infobox_Disease
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DiseasesDB = 33904
ICD10 =
ICD9 =
ICDO = 9678/3
OMIM =
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MeshID = D054685

Primary effusion lymphoma (PEL) is a B-cell lymphoma. [DorlandsDict|nine/10098969|primary effusion lymphoma]

Causes

It is caused by Kaposi's sarcoma-associated herpesvirus (KSHV), also known as human herpesvirus 8 (HHV-8). [cite journal |author=Cesarman E, Chang Y, Moore PS, Said JW, Knowles DM |title=Kaposi's sarcoma-associated herpesvirus-like DNA sequences in AIDS-related body-cavity-based lymphomas |journal=N. Engl. J. Med. |volume=332 |issue=18 |pages=1186–91 |year=1995 |month=May |pmid=7700311 |doi= |url=http://content.nejm.org/cgi/pmidlookup?view=short&pmid=7700311&promo=ONFLNS19] [cite journal |author=Staudt MR, Kanan Y, Jeong JH, Papin JF, Hines-Boykin R, Dittmer DP |title=The tumor microenvironment controls primary effusion lymphoma growth in vivo |journal=Cancer Res. |volume=64 |issue=14 |pages=4790–9 |year=2004 |month=July |pmid=15256448 |doi=10.1158/0008-5472.CAN-03-3835 |url=http://cancerres.aacrjournals.org/cgi/pmidlookup?view=long&pmid=15256448]

In most cases, the lymphoma cells are also infected with Epstein Barr virus (EBV).cite journal |author=Fan W, Bubman D, Chadburn A, Harrington WJ, Cesarman E, Knowles DM |title=Distinct subsets of primary effusion lymphoma can be identified based on their cellular gene expression profile and viral association |journal=J. Virol. |volume=79 |issue=2 |pages=1244–51 |year=2005 |month=January |pmid=15613351 |pmc=538532 |doi=10.1128/JVI.79.2.1244-1251.2005 |url=http://jvi.asm.org/cgi/pmidlookup?view=long&pmid=15613351]

PEL most commonly arises in patients with underlying immunodeficiency, such as AIDS. [cite journal |author=Boshoff C, Weiss R |title=AIDS-related malignancies |journal=Nat. Rev. Cancer |volume=2 |issue=5 |pages=373–82 |year=2002 |month=May |pmid=12044013 |doi=10.1038/nrc797 |url=http://dx.doi.org/10.1038/nrc797] [cite journal |author=Yarchoan R, Tosato G, Little RF |title=Therapy insight: AIDS-related malignancies--the influence of antiviral therapy on pathogenesis and management |journal=Nat Clin Pract Oncol |volume=2 |issue=8 |pages=406–15; quiz 423 |year=2005 |month=August |pmid=16130937 |doi= |url=]

The condition can exist in the absence of HHV-8 and HIV, though this is rare.cite journal |author=Youngster I, Vaisben E, Cohen H, Nassar F |title=An unusual cause of pleural effusion |journal=Age Ageing |volume=35 |issue=1 |pages=94–6 |year=2006 |month=January |pmid=16364944 |doi=10.1093/ageing/afj009 |url=http://ageing.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=16364944]

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".

A case has been described that was positive for CD38, CD71 and CD30.cite web |url=http://www.healthsystem.virginia.edu/internet/pathology/casestudies/heme/k98_3.cfm |title=Case 98-3 - AIDS-Related Primary Effusion Lymphoma |format= |work= |accessdate=]

History

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

Prognosis

It is generally resistant to cancer chemotherapy drugs that are active against other lymphomas, and carries a poor prognosis.cite journal |author=Chen YB, Rahemtullah A, Hochberg E |title=Primary effusion lymphoma |journal=Oncologist |volume=12 |issue=5 |pages=569–76 |year=2007 |month=May |pmid=17522245 |doi=10.1634/theoncologist.12-5-569 |url=http://theoncologist.alphamedpress.org/cgi/pmidlookup?view=long&pmid=17522245]

Sirolimus has been proposed as a treatment option.cite journal |author=Sin SH, Roy D, Wang L, "et al" |title=Rapamycin is efficacious against primary effusion lymphoma (PEL) cell lines in vivo by inhibiting autocrine signaling |journal=Blood |volume=109 |issue=5 |pages=2165–73 |year=2007 |month=March |pmid=17082322 |pmc=1801055 |doi=10.1182/blood-2006-06-028092 |url=http://www.bloodjournal.org/cgi/pmidlookup?view=long&pmid=17082322]

References