Primary effusion lymphoma
Overview
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.
Symptoms
The list of signs and symptoms mentioned in various sources for Primary effusion lymphoma includes the 2 symptoms listed below: * Shortness of breath * Build up of fluid inside body cavity Note that Primary effusion lymphoma symptoms usually refers to various symptoms known to a patient, but the phrase Primary effusion lymphoma signs may refer to those signs only noticable by a doctor.
Causes
It is caused by Kaposi's sarcoma-associated herpesvirus (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, such as AIDS. The condition can exist in the absence of HHV-8 and HIV, though this is rare.
Prognosis
It is generally resistant to cancer chemotherapy drugs that are active against other lymphomas, and carries a poor prognosis. Sirolimus has been proposed as a treatment option.