Intraclonal diversity in a Sezary syndrome with a differential response to 2-deoxycoformycin of the two lymphoma cell populations

Br J Haematol. 2002 Dec;119(3):629-33. doi: 10.1046/j.1365-2141.2002.03889.x.

Abstract

We report a case of Sezary syndrome with two abnormal CD4+ T-cell populations detected in the peripheral blood by flow cytometry immunophenotyping and DNA cell content, suggesting a biclonal T-cell lymphoproliferative disorder. Despite these findings, molecular analysis of the T-cell receptor genes was consistent with a monoclonal T-cell proliferation, supporting the existence of intraclonal diversity rather than a true biclonal disease. The patient achieved a transient response with 2-deoxycoformycin, with a selective decrease of the larger/hyperploid T-cell population; later on, an increased representation of this T-cell population was observed concomitantly with clinical relapse.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Antibiotics, Antineoplastic / therapeutic use*
  • CD4-Positive T-Lymphocytes / immunology*
  • Cell Division
  • Flow Cytometry
  • Humans
  • Immunophenotyping / methods
  • Male
  • Pentostatin / therapeutic use*
  • Phenotype
  • Sezary Syndrome / drug therapy*
  • Sezary Syndrome / immunology
  • Skin Neoplasms / drug therapy*
  • Skin Neoplasms / immunology
  • T-Lymphocyte Subsets / immunology*

Substances

  • Antibiotics, Antineoplastic
  • Pentostatin