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 ?
Human Herpesvirus 8(HHV-8) Associated Peritoneal Primary Effusion Lymphoma (PEL) in Two HIV-Negative Elderly Patients.
HHV-8/KSHV-Negative and CD20 Positive Primary Effusion Lymphoma Successfully Treated by Pleural drainage Followed by Chemoteraphy Containing Rituximab.
Human herpesvirus-8-associated lymphoma of the bowel in human immunodeficiency virus-positive patients without history of primary effusion lymphoma.
Kaposi's sarcoma-associated herpesvirus/human herpesvirus type 8-positive solid lymphomas: a tissue-based variant of primary effusion lymphoma.
DLBCL Plasmablastic lymphoma Large B-cell lymphoma arising in HHV8-associated multicentric Castleman disease Primary effusion lymphoma Borderline cases B-cell lymphoma, unclassifiable, with features intermediate between DLBCL and Burkitt lymphoma B-cell lymphoma, unclassifiable, with features intermediate between DLBCL and classical Hodgkin lymphoma Abbreviations: ALK, anaplastic lymphoma kinase; CNS, central nervous system; EBV, Epstein-Barr virus; HHV8, human herpesvirus 8.
Human herpesvirus 8 in primary effusion lymphoma in an HIV-seronegative male: a case report.
Treatment of primary effusion lymphoma with highly active antiviral therapy in the setting of HIV infection.
Distribution of human herpesvirus-8 latently infected cells in Kaposi's sarcoma, multicentric Castleman's disease, and primary effusion lymphoma.
Differential viral protein expression in Kaposi's sarcoma-associated herpesvirus-infected diseases: Kaposi's sarcoma, primary effusion lymphoma, and multicentric Castleman's disease.
HHV-8/KSHV viral genomes are not present in pyothorax-associated lymphoma, a further feature that contrasts with primary effusion lymphoma.
4,73,80) Primary effusion lymphoma cells appear more uniform in histologic sections than in cytocentrifuge preparations.
Expression of potentially oncogenic HHV-8 genes in an EBV-negative primary effusion lymphoma occurring in an HIV-seronegative patient.