primary effusion lymphoma


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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 ?
Primary Effusion Lymphoma: Cytopathologic Diagnosis Using In Situ Molecular Genetic Analysis for Human Herpesvirus 8.
Primary Effusion lymphoma in Two HIV-Negative Patients Successfully Treated with Pleurodesis as First-line Theraphy.
Herpesvirus 8 inclusions in primary effusion lymphoma: report of a unique case with T-cell phenotype.
Distinct cellular origins of primary effusion lymphoma with and without EBV infection.
Prognosis in primary effusion lymphoma is associated with the number of body cavities involved.
Comparison of human herpes virus 8 related primary effusion lymphoma with human herpes virus 8 unrelated primary effusion lymphoma-like lymphoma on the basis of HIV: report of 2 cases and review of 212 cases in the literature.
Primary effusion lymphoma is a HHV8-related lymphoma.
Primary effusion lymphoma. In: Pathology and Genetics of Haematopoietic and Lymphoid Tissues.
Distribution of human herpesvirus-8 latently infected cells in Kaposi's sarcoma, multicentric Castleman's disease, and primary effusion lymphoma. Proc Natl Acad Sci U S A.
Prognostic factors and outcome of human herpesvirus 8-associated primary effusion lymphoma in patients with AIDS.
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.[6] In the latter case, neoplastic lymphoid cells expressed various T-cell-specific antigens, including CD2, CD3, CD5, and CD7, and no B-cell markers, and both T-cell receptor and immunoglobulin gene rearrangements.[6] In our case, several cytologic and phenotypic features could be suggestive of ALCL.