Primary effusion lymphoma (PEL)

PRIMARY EFFUSION LYMPHOMA (PEL)
Aetiology : it occurs in patients with HIV

infection

HHV-8 / KSHV ⁺ immunoblasts with plasmacytoid cytoplasm. Most PELs have concomitant HHV-4 / EBV infection and these viral double positive cells harbour mutated rearranged Ig genes, suggesting that they originate from germinal centre or post-germinal centre B-cells, suggesting that EBV is an important pathogenetic cofactor, although other as yet unidentified cofactors, such as cellular genetic alterations, are also likely to play a role. Within the KSHV⁺ PELs, the effect of EBV is more subtle but nevertheless clear^ref

HHV-4 / EBV ⁺ PELs (most) are derived from germinal centre or post-germinal centre B-cells
EBV^- PELs may originate from either germinal/post-germinal centre or naive B-cells^ref

⁺

^ref

Symptoms & signs : lymphomatous effusions primarily body cavities and occasionally extranodal sites without the presence of a solid tumor
Laboratory examinations : the tumor cells usually do not express immunoglobulin (Ig) and B-cell markers but express syndecan-1 (CD138)^ref and harbor hypermutated rearranged immunoglobulin genes^ref, suggesting that they originate from germinal center or postgerminal center B cells^ref

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