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[Plasma cell leukemia (IgA-lambda type) with polyclonal hypergammaglobulinemia]

T Hyodo1, K Iwamasa, S Nakatani

  • 1First Department of Internal Medicine, Ehime University School of Medicine.

[Rinsho Ketsueki] the Japanese Journal of Clinical Hematology
|October 31, 1998
PubMed
Summary

This study reports a rare case of plasma cell leukemia with polyclonal hypergammaglobulinemia in an elderly woman. Despite achieving remission with chemotherapy, the patient succumbed to invasive aspergillosis.

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Area of Science:

  • Hematology
  • Oncology
  • Immunology

Background:

  • Plasma cell leukemia (PCL) is a rare and aggressive malignancy characterized by the proliferation of malignant plasma cells.
  • Polyclonal hypergammaglobulinemia, an increase in multiple immunoglobulin classes, is typically associated with reactive conditions, not plasma cell neoplasms.

Observation:

  • An 84-year-old woman presented with fever and lymphadenopathy, exhibiting a high white blood cell count with a significant proportion of atypical plasma cells.
  • Laboratory findings included elevated lactate dehydrogenase (LDH) and interleukin-6 (IL-6), alongside a monoclonal IgA-lambda band and polyclonal hypergammaglobulinemia.

Findings:

  • The patient was diagnosed with plasma cell leukemia (IgA-lambda type) based on immunofixation-electrophoresis and Southern blotting analysis.

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  • Treatment with combination chemotherapy for acute lymphocytic leukemia resulted in complete remission.
  • Genetic analysis revealed rearranged monoclonal bands in JH and J lambda genes, confirming the plasma cell origin.

    • Implications:

    • This case represents the first documented instance of plasma cell leukemia presenting with polyclonal hypergammaglobulinemia.
    • The findings challenge conventional understanding of immunoglobulin profiles in plasma cell malignancies.
    • Further research is warranted to elucidate the mechanisms underlying this rare presentation and its impact on patient outcomes.