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Hairy cell leukemia variant

P Dunn1, L Y Shih, Y S Ho

  • 1Department of Internal Medicine, Chang Gung Memorial Hospital, Taipei, Taiwan, China.

Acta Haematologica
|January 1, 1995
PubMed

Insights

This case study details a rare variant of hairy cell leukemia in a 59-year-old man, highlighting its aggressive nature and resistance to standard treatments. The patient ultimately succumbed to sepsis despite initial partial remission following splenic irradiation.

Area of Science:

  • Hematology
  • Oncology
  • Immunophenotyping

Background:

  • Hairy cell leukemia (HCL) is a rare B-cell chronic lymphoid leukemia.
  • HCL variant (HCL-v) is a distinct subtype with unique morphological and immunophenotypic features.
  • Understanding HCL-v is crucial for developing targeted therapeutic strategies.

Observation:

  • A 59-year-old male presented with lymphocytosis and massive splenomegaly.
  • Abnormal lymphocytes exhibited a high nucleus-to-cytoplasm ratio, prominent nucleolus, and characteristic cytoplasmic projections.
  • Immunophenotyping was negative for CD5 and CD25, consistent with B-cell leukemia.

Findings:

  • Cytogenetic analysis revealed complex chromosomal abnormalities, including rearrangements of chromosomes 5, 6, 7, and 22.
  • The patient showed no response to conventional chemotherapy (chlorambucil, cyclophosphamide, vincristine, prednisolone).
  • Splenic irradiation resulted in a partial remission, but the disease progressed.

Implications:

  • This case underscores the aggressive clinical course and treatment resistance often seen in HCL variant.
  • The complex cytogenetics may contribute to the refractory nature of the disease.
  • Further research into novel therapeutic targets for HCL variant is warranted to improve patient outcomes.

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