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Diseases » Primary effusion lymphoma » Summary

What is Primary effusion lymphoma?

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What is Primary effusion lymphoma?

Primary effusion lymphoma: A cancerous proliferation of lymphocytic B-cells caused by the human herpesvirus 8 (HHV-8, Kaposi's sarcoma-associated herpesvirus). It is more prevalent in immunodeficient people such as AIDS patients. The cancer tends to occur in the lining of body cavities such as the pericardium and peritoneum. The cancerous cells are detected in the fluid secreted from the lining of the cavity.
Primary effusion lymphoma: An aggressive non-Hodgkin's B-cell lymphoma composed of large cells, presenting as a serous effusion without detectable tumor masses. It is universally associated with human herpes virus 8 (HHV-8)/Kaposi sarcoma herpes virus (KSHV) [HHV-8/KSHV]. It mostly occurs in the setting of immunodeficiency; most cases have been reported in HIV positive patients. The most common sites of involvement are the pleural, pericardial, and peritoneal cavities. The prognosis is extremely unfavorable. -- 2004
Source - Diseases Database

Primary effusion lymphoma is listed as a "rare disease" by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH). This means that Primary effusion lymphoma, or a subtype of Primary effusion lymphoma, affects less than 200,000 people in the US population.
Source - National Institutes of Health (NIH)

Ophanet, a consortium of European partners, currently defines a condition rare when it affects 1 person per 2,000. They list Primary effusion lymphoma as a "rare disease".
Source - Orphanet

Primary effusion lymphoma: Introduction