Causes

It is caused by Kaposi's sarcoma-associated herpesvirus

Kaposi's sarcoma-associated herpesvirus

Kaposi's sarcoma-associated herpesvirus is one of seven currently known human cancer viruses, or oncoviruses. It is also the eighth human herpesvirus; its formal name according to the International Committee on Taxonomy of Viruses is HHV-8. Like other herpesviruses, its informal name is used...

 (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 immunodeficiency

Immunodeficiency

Immunodeficiency is a state in which the immune system's ability to fight infectious disease is compromised or entirely absent. Immunodeficiency may also decrease cancer immunosurveillance. Most cases of immunodeficiency are acquired but some people are born with defects in their immune system,...

, such as AIDS

AIDS

Acquired 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 pericardium

Pericardium

The pericardium is a double-walled sac that contains the heart and the roots of the great vessels.-Layers:...

; another name for PEL is "body cavity lymphoma".

A case has been described that was positive for CD38

CD38

CD38 , 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 CD30

CD30

CD30, 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 chemotherapy

Chemotherapy

Chemotherapy is the treatment of cancer with an antineoplastic drug or with a combination of such drugs into a standardized treatment regimen....

 drugs that are active against other lymphomas, and carries a poor prognosis.

Sirolimus

Sirolimus

Sirolimus , 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.