Plasma Cell Leukemia (IgA-λ type) with Polyclonal Hypergammaglobulinemia

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  • 多クローン性高γグロブリン血症を伴った形質細胞性白血病（IgA-λ型）

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An 84-year-old woman was admitted to Ehime University hospital because of fever and generalized swelling of lymph nodes. On admission, she did not have any bone diseases. The laboratory data included a WBC count of 60,900/μl, with 80.5% atypical plasma cells in the peripheral blood and 26.4% in the bone marrow. The patient's serum total protein was 9.3 g/dl with increased polyclonal γ-globulin (62.4%). Serum levels of LDH (1,986 IU/l) and IL-6 (34.3 pg/dl) were also elevated. Immunofixation-electrophoresis detected a monoclonal band defined as IgA-λ type, with a broad band of polyclonal immunoglobulin. Southern blotting analysis demonstrated rearranged monoclonal bands in the JH and J λ genes. Based on these findings, plasma cell leukemia (IgA-λ type) was diagnosed. The patient was treated with combination chemotherapy for acute lymphocytic leukemia and achieved complete remission. However, she died of aspergillus sepsis two months after admission. After chemotherapy, the patients IgG and IgM levels normalized but IgA still showed a slight increase. IL-6 also decreased, from 34.3 pg/dl to 10.2 pg/dl. To our knowledge, this report is the first in the literature concerning a case of plasma cell leukemia with polyclonal hypergammanglobulinemia.

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 39 (9), 680-685, 1998

    The Japanese Society of Hematology

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