Primary effusion lymphoma (PEL)

PRIMARY EFFUSION LYMPHOMA (PEL)

Aetiology : it occurs in patients with HIV infection

HHV-8 / KSHV ⁺ immunoblasts with plasmacytoid cytoplasm

HHV-4 / EBV ⁺ PELs (most) : these viral double positive cells harbour mutated rearranged Ig genes, suggesting that they originate from germinal centre or post-germinal centre B-cells. Within the KSHV⁺ PELs, the effect of EBV is more subtle but nevertheless clear^ref
EBV^- PELs may originate from either germinal/post-germinal centre or naive B-cells^ref

⁺

^ref

Pathogenesis : PEL cells were able to produce tumors and ascites very efficiently with infiltration of cells in various organs of T-, B- and NK-cell knock-out NOD/SCID/g_c(null) (NOG) mice within 3 weeks. In contrast, PEL cells formed small tumors at inoculated sites in T- and B-cell knock-out NOD/SCID mice with NK-cells while completely failing to infiltrate into various organs. Immunosupression of NOD/SCID by treatment with an antimurine TM-b₁ antibody, which transiently abrogates NK cell activity in vivo, resulted in enhanced tumorigenicity and organ infiltration in comparison with non-treated NOD/SCID mice. Activated human NK cells inhibited tumor growth and infiltration in NOG mice. NK cells play an important role in growth and infiltration of PEL cells, and activated NK cells could be a promising immunotherapeutic tool against tumor or virus-infected cells either alone or in combination with conventional therapy. The rapid and efficient engraftment of PEL cells in NOG mice also suggests that this new animal model could provide a unique opportunity to understand and investigate the mechanism of pathogenesis and malignant cell growth^ref
Symptoms & signs : lymphomatous effusions primarily in 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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