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[Hairy cell leukemia, Japanese variant, successfully treated with cladribine]

Mutsuko Miyazaki1, Akihiko Taguchi, Shizu Sakuragi

  • 1Division of Hematology, Department of Medicine, Yamaguchi Prefecture Central Hospital.

[Rinsho Ketsueki] the Japanese Journal of Clinical Hematology
|June 18, 2004
PubMed

Insights

Hairy cell leukemia, Japanese variant, presents with lymphocytosis and splenomegaly. Cladribine effectively induced remission in a patient unresponsive to interferon-alpha therapy.

Area of Science:

  • Hematology
  • Oncology
  • Immunology

Background:

  • Hairy cell leukemia (HCL) is a rare B-cell chronic lymphoid leukemia.
  • The Japanese variant of HCL exhibits distinct clinical and immunophenotypic features.

Observation:

  • A 67-year-old male presented with lymphocytosis and significant splenomegaly.
  • Peripheral blood and bone marrow revealed abnormal lymphocytes with cytoplasmic projections, negative for tartrate-resistant acid phosphatase.
  • Flow cytometry showed increased IgM, kappa-positive B cells expressing CD11c, CD19, CD20, and FMC7, but negative for CD5, CD10, and CD25.

Findings:

  • The patient was diagnosed with Hairy Cell Leukemia, Japanese variant.
  • Initial treatment with interferon-alpha yielded limited success.
  • Subsequent cladribine therapy resulted in a complete and sustained remission for 8 months.

Implications:

  • This case highlights cladribine as a highly effective treatment for Hairy Cell Leukemia, Japanese variant.
  • The findings support cladribine's role in managing refractory or relapsed HCL.
  • Understanding the immunophenotypic profile is crucial for accurate diagnosis and treatment selection.

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