Primary effusion lymphoma

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Primary effusion lymphoma
Classification and external resources
Specialty Hematology and oncology
ICD-O M9678/3
DiseasesDB 33904
Patient UK Primary effusion lymphoma
MeSH D054685
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Primary effusion lymphoma (PEL) is a B-cell lymphoma, presenting with a malignant effusion without a tumor mass.[1][2]:879-881

Causes

PEL most commonly arises in patients with underlying immunodeficiency, such as AIDS.[3][4] PEL is caused by Kaposi's sarcoma-associated herpesvirus (KSHV), also known as human herpesvirus 8 (HHV-8).[5][6] In most cases, the lymphoma cells are also infected with Epstein Barr virus (EBV).[7]

The condition can exist in the absence of HHV-8 and HIV, though this is rare.[8]

Presentation

PEL is unusual in that the majority of cases arise in body cavities, such as the pleural space or the pericardium; another name for PEL is "body cavity lymphoma".

The immunophenotype : CD45+ (95%), CD20-, CD79a-, PAX5-, CD30+, CD38+, CD138+ and EMA+.[2]:879-881 Staining the nucleus for HHV-8 LANA may be helpful.[2]:879-881

History

It was recognized as a unique type of lymphoma only after the discovery of KSHV in 1994.

Prognosis

It is generally resistant to cancer chemotherapy drugs that are active against other lymphomas, and carries a poor prognosis.[9]

Sirolimus has been proposed as a treatment option.[10]

See also

References

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