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Immunoglobulin Gene Sequence Analysis In Chronic Lymphocytic Leukemia: From Patient Material To Sequence Interpretation
09:02

Immunoglobulin Gene Sequence Analysis In Chronic Lymphocytic Leukemia: From Patient Material To Sequence Interpretation

Published on: November 26, 2018

Simultaneous manifestation of chronic lymphocytic leukemia (CLL) and hairy cell leukemia (HCL)

Lubomir Sokol1, Steven J Agosti

  • 1Department of Interdisciplinary Oncology, University of South Florida, H Lee Moffitt Cancer Center and Research Institute, Tampa, Florida, USA.

American Journal of Hematology
|February 3, 2004
PubMed

Insights

This case study details a rare instance of simultaneous chronic lymphocytic leukemia (CLL) and hairy cell leukemia (HCL) in an 83-year-old male. Flow cytometry identified two distinct B-cell clones, one for each leukemia, presenting unique diagnostic and therapeutic challenges.

Area of Science:

  • Hematology
  • Oncology
  • Immunology

Background:

  • Chronic lymphocytic leukemia (CLL) and hairy cell leukemia (HCL) are distinct mature B-cell malignancies.
  • Simultaneous occurrence of CLL and HCL is exceptionally rare, posing diagnostic and therapeutic complexities.

Observation:

  • An 83-year-old male presented with symptoms suggestive of CLL, including lymphocytosis and lymphadenopathy.
  • Flow cytometry revealed two separate abnormal B-cell clones: one consistent with HCL (CD20+, CD11c+, CD103+, CD25+, kappa+) and another with CLL (CD19+, CD20+, CD23+, CD5+, lambda+).
  • Bone marrow analysis later showed bright lambda light chain expression on HCL cells, differing from initial peripheral blood findings.

Findings:

  • The presence of two independent malignant B-cell clones was confirmed by distinct immunophenotypes and differing immunoglobulin light chain expression (kappa for HCL, lambda for CLL).
  • The HCL clone remained largely refractory to chemotherapy, persisting in the bone marrow at a stable low percentage (7-10%).
  • The CLL clone responded well to combination chemotherapy (fludarabine, cytoxan) and subsequent rituximab monotherapy.

Implications:

  • This case highlights the importance of comprehensive flow cytometry in diagnosing B-cell malignancies, especially when unusual presentations occur.
  • The distinct behavior and treatment response of the two clones underscore the heterogeneity within lymphoid neoplasms.
  • Further research into the pathogenesis and optimal management of co-occurring lymphoid leukemias is warranted.

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