primary effusion lymphoma


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primary effusion lymphoma

a B cell lymphoma associated with human herpesvirus 8 infection, characterized by the occurrence of lymphomatous effusions in body cavities without the presence of a solid tumor.

primary effusion lymphoma

A very rare, high-grade human herpes virus 8- (HHV-8) associated large-cell lymphoma of body cavities with evidence of EBV infection, which primarily affects immunocompromised patients, especially those with HIV disease.

Clinical findings
Fever of unknown origin, shortness of breath, weakness, cough.
 
Risk factors
HCV, HIV, immunocompromise, elderly, asbestos exposure, solid organ transplantation.

Sites
Pleura, pericardium, or peritoneum, often with effusion.
 
Management
CHOP

Prognosis
Most patients die from the disease.

primary effusion lymphoma

A non-Hodgkin B-cell lymphoma that typically arises in body cavities such as the pleural, peritoneal, or pericardial spaces. It is caused by Kaposisarcoma herpesvirus (human herpes virus 8) and is usually found in patients with advanced immune suppression.
Synonym: body cavity lymphoma
See also: lymphoma
References in periodicals archive ?
Kaposi's sarcoma-associated herpesvirus/human herpesvirus type 8-positive solid lymphomas: a tissue-based variant of primary effusion lymphoma.
KSHVpositive solid lymphomas represent an extra-cavitary variant of primary effusion lymphoma.
Complete remission of a primary effusion lymphoma with antiretroviral therapy.
Differential viral protein expression in Kaposi's sarcoma-associated herpesvirus-infected diseases: Kaposi's sarcoma, primary effusion lymphoma, and multicentric Castleman's disease.
Primary effusion lymphoma in pleural and pericardial cavities with multiple solid nodal and extranodal involvement in a human immunodeficiency virus-positive patient.
Association of Kaposi's sarcoma-associated herpesvirus-positive primary effusion lymphoma with expression of the CD138/syndecan-1 antigen.
Both the B-cell antigen expression and lack of aberrant T-cell expression in pyothorax-associated lymphoma contrast with that observed in primary effusion lymphoma.
Human Immunodeficiency Virus-Associated Gastrointestinal Tract Neoplasms in Southern Africa Mesenchymal Epstein-Barr virus-associated smooth muscle tumors Kaposi sarcoma Lymphoid Hodgkin lymphoma Non-Hodgkin lymphoma Burkitt lymphoma Diffuse large B-cell lymphoma, not otherwise specified Mucosa-associated lymphoid tissue lymphoma Plasmablastic lymphoma Posttransplant lymphoproliferative disorder Primary effusion lymphoma Epithelial Anal intraepithelial neoplasia/squamous cell carcinoma Table 2.
Primary effusion lymphoma most commonly involves pleural, pericardial, and peritoneal cavities and presents without detectable tumor masses or lymphadenopathy.
Primary effusion lymphoma exhibiting a T-cell phenotype was reported only once, in an HIV-seropositive male patient.