HIV- and HHV-8-negative Primary Effusion Lymphoma Harboring the Dual Rearrangement of Antigen Receptor Genes and a Translocation of bcl-6

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Primary effusion lymphoma (PEL) is a rare type of lymphoma occurring in HIV-seropositive persons. Since the tumor cells frequently harbor human herpes virus (HHV)-8 DNA, the virus is thought to be causative. We here report an instructive case of PEL in an HIV-seronegative elderly. The neoplastic cells in the fluid showed unusual morphology with multi-lobulated nuclei. They expressed CD19, CD20, CD79a and SmIgD/Mκ, but not CD3, CD30 or CD23. The IgH gene was clonally rearranged based on the results of Southern blotting and PCR for CDR3. In addition to the IgH clonality, the TCRγ-chain gene was also in the clonal rearrangements. Neither HHV8 nor EBV was detected in the cells. The bcl-6 and IgH chimeric transcripts were evident. Conclusively, our case suggests the following two points: first, the bcl-6/IgH translocation may be associated with the pathogenesis of unusual PEL without HIV or HHV-8; and second, unusual PEL may be an extra-nodal spectrum of diffuse large-cell lymphoma with neoplastic cells harboring the bcl-6/IgH translocation and dual rearrangements of antigen receptor genes.
長崎大学医学部の論文