Causes
It is caused by
Kaposi's sarcoma-associated herpesvirusKaposi's sarcoma-associated herpesvirus is the eighth human herpesvirus; its formal name according to the International Committee on Taxonomy of Viruses is HHV-8...
(KSHV), also known as human herpesvirus 8 (HHV-8).
In most cases, the lymphoma cells are also infected with Epstein Barr virus (EBV).
PEL most commonly arises in patients with underlying
immunodeficiencyImmunodeficiency is a state in which the immune system's ability to fight infectious disease is compromised or entirely absent. Most cases of immunodeficiency are acquired but some people are born with defects in the immune system, or primary immunodeficiency...
, such as
AIDSAcquired immune deficiency syndrome or acquired immunodeficiency syndrome is a disease of the human immune system caused by the human immunodeficiency virus ....
.
The condition can exist in the absence of HHV-8 and HIV, though this is rare.
Presentation
PEL is unusual in that the majority of cases arise in body cavities, such as the pleural space or the
pericardiumThe pericardium is a double-walled sac that contains the heart and the roots of the great vessels.-Layers:There are two layers to the pericardial sac: the fibrous pericardium and the serous pericardium...
; another name for PEL is "body cavity lymphoma".
A case has been described that was positive for
CD38CD38 , also known as cyclic ADP ribose hydrolase is a glycoprotein found on the surface of many immune cells , including CD4+, CD8+, B and natural killer cells...
, CD71 and
CD30CD30, also known as TNFRSF8, is a cell membrane protein of the tumor necrosis factor receptor family and tumor marker.This receptor is expressed by activated, but not by resting, T and B cells. TRAF2 and TRAF5 can interact with this receptor, and mediate the signal transduction that leads to the...
.
Prognosis
It is generally resistant to cancer
chemotherapyChemotherapy, in its most general sense, is the treatment of disease by chemicals especially by killing micro-organisms or cancerous cells. In popular usage, it refers to antineoplastic drugs used to treat cancer or the combination of these drugs into a cytotoxic standardized treatment regimen...
drugs that are active against other lymphomas, and carries a poor prognosis.
SirolimusSirolimus , also known as rapamycin, is an immunosuppressant drug used to prevent rejection in organ transplantation; it is especially useful in kidney transplants. A macrolide, sirolimus was first discovered as a product of the bacterium Streptomyces hygroscopicus in a soil sample from Easter...
has been proposed as a treatment option.