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A Unique Hairy Cell Leukemia Variant.

Charles Jian1, Cyrus C Hsia2

  • 1Schulich School of Medicine and Dentistry, Western University, London Health Sciences Centre, London, Ont., Canada.

Case Reports in Oncology
|July 28, 2016
PubMed
Summary
This summary is machine-generated.

Hairy cell leukemia variant (HCL-v) is a more aggressive B-cell lymphoma than HCL, often resistant to standard treatments. Early identification through morphology and flow cytometry is crucial for effective patient management and therapy selection.

Keywords:
B-cell lymphoid leukemiaHairy cell leukemia variant

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Area of Science:

  • Hematology
  • Oncology
  • Immunology

Background:

  • Hairy cell leukemia (HCL) is a rare mature B-cell neoplasm.
  • HCL variant (HCL-v) presents with distinct clinical and morphological features.
  • Distinguishing HCL from HCL-v is critical for appropriate therapeutic strategies.

Observation:

  • A 65-year-old woman presented with splenomegaly, lymphocytosis, and atypical B-cell morphology.
  • Initial diagnosis of HCL was made, but the patient showed resistance to cladribine treatment.
  • Flow cytometry confirmed a monoclonal B-cell population.

Findings:

  • The patient's disease progressed despite standard HCL therapy, indicating HCL-v.
  • HCL-v demonstrated resistance to purine nucleoside analogs and required multi-agent chemotherapy (CHOP-R) and splenectomy.
  • Morphological analysis revealed atypical features, including large nucleoli and convoluted nuclei.

Implications:

  • HCL-v is a more aggressive lymphoma than HCL, characterized by significant splenomegaly and higher lymphocyte counts.
  • Prompt recognition of HCL-v through clinical presentation, morphology, and flow cytometry is essential for guiding treatment.
  • Patients with HCL-v necessitate more aggressive treatment regimens and closer monitoring compared to classical HCL.