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[Multiple myeloma of the IgD-lambda type invading CNS].

Kana Kitamura1, Jin Takeuchi, Emiko Kanbe

  • 1Division of 1st Internal Medicine, Department of Internal Medicine, Nihon University School of Medicine.

[Rinsho Ketsueki] the Japanese Journal of Clinical Hematology
|November 24, 2004
PubMed
Summary

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This case study highlights a rare IgD-lambda multiple myeloma presentation with extramedullary disease. Despite initial treatment, the patient experienced central nervous system relapse and generalized spread, emphasizing the aggressive nature of this myeloma subtype.

Area of Science:

  • Hematology
  • Oncology

Background:

  • Multiple myeloma is a plasma cell malignancy.
  • IgD myeloma is a rare subtype, often presenting with extramedullary disease.

Observation:

  • A 52-year-old woman presented with an abdominal mass, pancytopenia, and elevated LDH and IgD.
  • Bone marrow biopsy revealed 63.8% plasma cells with IgD-lambda M-protein, diagnosing multiple myeloma.
  • Despite VAD therapy, extramedullary masses persisted and new ones emerged.

Findings:

  • Combination chemotherapy (ROAD) and thalidomide were initiated, followed by radiation therapy.
  • The patient developed central nervous system (CNS) relapse with 93% plasma cells in cerebrospinal fluid.
  • Intrathecal chemotherapy with MTX and prednisolone temporarily controlled CNS disease.

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Implications:

  • This case underscores the challenges in managing IgD-lambda multiple myeloma, particularly extramedullary and CNS involvement.
  • Aggressive treatment strategies may be necessary for this rare and aggressive subtype.
  • Further research is needed to improve outcomes for patients with IgD myeloma and CNS relapse.