症例報告 IgG-κ型M蛋白血症を認めたHIV,HHV-8/KSHV陰性Primary effusion lymphoma

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A 93-year-old man was referred to our hospital due to shortness of breath. Computed tomographic scans revealed bilateral pleural effusion, but lymphadenopathy was not found. Pleural effusion specimens contained numerous atypical cells possessing indented nuclei and moderate amounts of basophilic cytoplasm containing numerous small round vacuoles. Flow cytometric analyses of the cell surface demonstrated that these cells were positive for CD19 and CD20. Southern blot analysis revealed a clonal rearrangement of the immunoglobulin heavy chain gene, and chromosome analysis showed near-tetraploid karyotype accompanied with structural aberrations. Human herpes virus (HHV) -8/Kaposi's sarcoma-associated herpes virus (KSHV) was not identified within the nuclei of the cells by immunostaining, and a serologic test for human immunodeficiency virus (HIV) was negative. The value of IgG was 2560 mg/dl. Furthermore, serum electrophoresis revealed the existence of an M-component of 36.1%, and serum immunoelectrophoresis showed that the component was a IgG-κ type monoclonal protein. Bence Jones proteinuria was not detected. On the other hand, the ratio of plasma cells in the bone marrow was 5.0%, and a bone lesion was not found. Base on these results, we diagnosed this patient with HIV-, HHV-8/KSHV- negative primary effusion lymphoma (PEL) . We concluded that the monoclonal proteins are produced in lymphoma cells because flow cytometric analyses showed that these cells express IgG and κ-chain within cytoplasm. To our knowledge, this is the first report of a PEL showing monoclonal proteinemia.
学校法人昭和大学・昭和医学会の論文

症例報告 IgG-κ型M蛋白血症を認めたHIV,HHV-8/KSHV陰性Primary effusion lymphoma

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