[Large granular lymphocyte leukemia containing oligoclonal EB viral DNA]

K Itoh1, T Inaba, S Murakami

  • 1Second Department of Internal Medicine, Kyoto Prefectural University of Medicine.

Insights

This study identifies a rare case of lymphoproliferative disorder of granular lymphocytes (LDGL) in a female patient. Analysis suggests multiple clones, with chromosome abnormalities aiding in diagnosis.

Area of Science:

  • Hematology
  • Immunology
  • Oncology

Background:

  • Lymphoproliferative disorder of granular lymphocytes (LDGL) is a rare hematologic malignancy.
  • Diagnosing LDGL can be challenging due to difficulties in demonstrating clonal origins.

Observation:

  • A 32-year-old female presented with abdominal fullness, jaundice, and edema.
  • Peripheral blood revealed leukocytosis with 84% leukemic cells; bone marrow showed 63.6% leukemic cells.
  • Leukemic cells expressed CD2, OKIa1, NKH-1, exhibited NK and ADCC activity, but lacked T cell and NK cell markers (CD3, CD4, CD8, CD16).
  • T cell receptor and immunoglobulin genes were in germline configuration, suggesting a non-T, non-B cell origin, likely NK cell lineage.

Findings:

  • Despite polyclonal Epstein-Barr Virus (EBV) genome analysis, a consistent chromosomal abnormality (47, XX, +3) was identified.
  • The findings suggest the presence of multiple clones, with chromosomal analysis detecting one clone.
  • The study highlights the utility of EBV genome analysis and chromosomal studies in diagnosing LDGL.

Implications:

  • This case expands the understanding of LDGL heterogeneity.
  • It underscores the importance of integrating cytogenetic and molecular analyses for accurate LDGL diagnosis.
  • The findings may guide future diagnostic strategies for similar hematologic malignancies.

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