Current concepts in primary effusion lymphoma and other effusion-based lymphomas.

[primary effusion lymphoma]

Primary effusion lymphoma (PEL ) is a human herpes virus 8 (HHV 8 )-positive large B-cell neoplasm that presents as an effusion with no detectable tumor in individuals with human immunodeficiency virus infection or other immune deficiencies . PEL is an aggressive neoplasm with a poor prognosis . PEL cells show diverse morphologies , ranging from immunoblastic or plasmablastic to anaplastic . The immunophenotype of PEL is distinct , but its lineage can be misdiagnosed if not assessed thoroughly . PEL cells usually express CD 4 5 , lack B- and T -cell-associated antigens , and characteristically express lymphocyte activation antigens and plasma cell-associated antigens . Diagnosis of PEL often requires the demonstration of a B-cell genotype . HHV 8 must be detected in cells to diagnose PEL . In most cases , PEL cells also harbor the Epstein-Barr virus (EBV ) genome . Similar conditions associated with HHV 8 but not effusion-based are called "extracavitary PELs ." PELs should be differentiated from HHV 8 -negative , EBV-positive , body cavity-based lymphomas in patients with long -standing chronic inflammation ; the latter can occur in tuberculous pleuritis , artificial pneumothorax , chronic liver disease and various other conditions . Despite their morphological similarity , these various lymphomas require different therapeutic strategies and have different prognostic implications . Correct diagnosis is essential to manage and predict the outcome of patients with PEL and related disorders .

Diseases
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Diseases presenting "neoplasm with a poor prognosis" symptom

carcinoma of the gallbladder

primary effusion lymphoma

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