Systemic Plasmacytosis.

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  • 全身性形質細胞増多症の１例

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We report a 35-year-old female who had a 4-year history of symptomatic cutaneous purpuric brownish macules that gradually developed on her face and back in association with a 4-year history of fever, general fatigue, enlargement of superficial lymph nodes, polyclonal hypergammaglobulinemia, and increased level of acute-phase proteins. Internists had failed to detect chronic infectious disease, collagen disease or other chronic inflammatory disorders. We made a diagnosis of systemic plasmacytosis based on histopathological findings, including hyperplasia of mature plasma cells in purpuric macules of the upper back and right axillary lymph node, and a significant increase in serum interleukin (IL)-6 level. Following administration of a moderate dose of prednisolone, the fever was reduced and the plasma level of acute-phase proteins was slightly decreased. Blockade of IL-6 signal transduction using humanized anti-IL-6 receptor antibody, a new therapeutic agent, is planned in the future. Systemic plasmacytosis is a benign plasma cell proliferation with polyclonal hyperglobulinemia, cutaneous manifestations of plasmacytosis and/or generalized plasmacytic lymphadenopathy, and it has clinical and histologic characteristics similar to those of multicentric Castleman’s disease.

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