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Related Experiment Videos

Hairy cell leukemia-variant--a case report.

Kirti Gupta1, Ahluwalia Jasmina, Pankaj Malhotra

  • 1Department of Haematology, PGIMER, Chandigarh.

Indian Journal of Pathology & Microbiology
|June 10, 2006
PubMed
Summary

Hairy-cell leukemia-variant (HCL-V) is a rare B-cell leukemia. This case study details its unique immunophenotype, resistance to standard treatments, and the potential palliative benefit of splenectomy.

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Area of Science:

  • Hematology
  • Oncology
  • Immunophenotyping

Background:

  • Hairy-cell leukemia-variant (HCL-V) is a rare B-cell neoplasm, comprising 10% of hairy-cell leukemia cases.
  • HCL-V presents with splenomegaly, lymphocytosis, and cytopenias, notably lacking monocytopenia.
  • Morphologically, circulating cells are intermediate between prolymphocytes and hairy cells.

Observation:

  • Immunophenotyping reveals a mature B-cell phenotype with CD11c and CD103 expression, but negative for CD25, distinguishing it from classic HCL.
  • Bone marrow and spleen histology show infiltration patterns similar to typical HCL.
  • A case of HCL-V in a 66-year-old male is presented, detailing bone marrow findings, immunophenotypic profile, and electron microscopic features.

Findings:

  • The patient's leukemia cells exhibited characteristic HCL-V morphology and immunophenotype.

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  • Bone marrow and spleen infiltration confirmed the diagnosis.
  • Patients with HCL-V demonstrate resistance to alkylating agents and alpha-interferon (á-IFN).

    • Implications:

    • Splenectomy may offer long-lasting partial responses and serves as a valuable palliative treatment option for HCL-V.
    • Understanding the distinct immunophenotype is crucial for accurate diagnosis and treatment planning.
    • Further research into novel therapeutic strategies for HCL-V is warranted due to treatment resistance.