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==Overview==
'''Primary effusion lymphoma''' (PEL) is a malignancy of B cells that is caused by [[Kaposi's sarcoma-associated herpesvirus]] (KSHV), also known as human herpesvirus 8 (HHV-8)<ref>Cesarman E, Chang Y, Moore PS, Said JW, Knowles DM. Kaposi's sarcoma-associated herpesvirus-like DNA sequences in AIDS-related body-cavity-based lymphomas. N Engl J Med 1995;332(18):1186-91.</ref>. In about 80% of cases, the lymphoma cells are also infected with [[Epstein Barr virus]] (EBV).  PEL is unusual in that the majority of cases arise in body cavities, such as the pleural space or the pericardium<ref>Staudt MR, Kanan Y, Jeong JH, Papin JF, Hines-Boykin R, Dittmer DP. The tumor microenvironment controls primary effusion lymphoma growth in vivo. Cancer Res 2004;64(14):4790-9.</ref>.
==Historical Perspective==
Primary effusion lymphoma was recognized as a unique type of lymphoma only after the discovery of KSHV in 1994.
PEL most commonly arises in patients with underlying immunodeficiency, such as AIDS[1]. It is caused by Kaposi's sarcoma-associated herpesvirus (KSHV).

Latest revision as of 20:06, 23 March 2016

 
 
 
 
 
 
 
 
 
B-cell neoplasms
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
CD5+
 
 
 
 
 
 
 
CD5-
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
CCND1+
 
 
 
CCND1-
 
 
 
 
DLBCL
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Pleomorphic MCL
 
 
 
DLBCL, NOS CD5+
 
CD10+
 
 
 
 
 
 
 
CD10-
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
DLBCL, NOS GCB type
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
BCL6+ IRF4/MUM1-
 
BCL6+ IRF4/MUM1+
 
BCL6- IRF4/MUM1+
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
DLBCL, NOS GCB type
 
Non-GCB
 
Post-GCB
 
 



Use of Immunophenotyping/Genetic Testing in Differential Diagnosis of Mature B-Cell and NK/T-Cell Neoplasms


 
 
 
 
 
 
 
 
 
B-cell neoplasms
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
CD5+
 
 
 
 
 
 
 
CD5-
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
CCND1+
 
 
 
CCND1-
 
 
 
 
DLBCL
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Pleomorphic MCL
 
 
 
DLBCL, NOS CD5+
 
CD10+
 
 
 
 
 
 
 
CD10-
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
DLBCL, NOS GCB type
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
BCL6+ IRF4/MUM1-
 
BCL6+ IRF4/MUM1+
 
BCL6- IRF4/MUM1+
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
DLBCL, NOS GCB type
 
Non-GCB
 
Post-GCB