Improvement of extrathymic T cell type of large granular lymphocyte (LGL) leukemia by cyclosporin A: the serum level of Fas ligand is a marker of LGL leukemia activity

Eur J Haematol. 2000 Oct;65(4):272-5. doi: 10.1034/j.1600-0609.2000.065004272.x.

Abstract

We report a case of gammadelta T-cell-type large granular lymphocyte (LGL) leukemia (CD3 +,CD8 +, CD57 +,TCR gammadelta+), which was accompanied by pure red cell aplasia, neutropenia and thrombocytosis. Southern blotting analysis of the T-cell receptor beta gene showed the germline configuration, but clonal TCR J gamma rearrangements were identified. These granular lymphocytes demonstrated non-major histocompatibility complex-restricted cytotoxicitity. The serum-soluble FasL (sFasL) concentration of this patient was very high, whereas the serum levels of tumor necrosis factor alpha (TNF-alpha), interferon gamma (IFN-gamma), interleukin-1 beta (IL-1beta), interleukin-2 (IL-2) and thrombopoietin were normal. After treatment with cyclosporin A, anemia and thrombocytosis were improved, and LGL and the elevated sFasL concentration decreased. These observations suggested that FasL may have played a role in the establishment of the clinical symptoms of this patient and could be useful as an indicator of disease activity.

Publication types

  • Case Reports

MeSH terms

  • CD3 Complex / blood
  • Cyclosporine / administration & dosage*
  • Humans
  • Immunophenotyping
  • Leukemia, Lymphoid / complications
  • Leukemia, Lymphoid / drug therapy*
  • Leukemia, T-Cell / complications
  • Leukemia, T-Cell / drug therapy*
  • Male
  • Middle Aged
  • Receptors, Antigen, T-Cell, gamma-delta / blood
  • Red-Cell Aplasia, Pure / drug therapy
  • Red-Cell Aplasia, Pure / etiology
  • Thrombocytosis / drug therapy
  • Thrombocytosis / etiology
  • Treatment Outcome
  • fas Receptor / blood

Substances

  • CD3 Complex
  • Receptors, Antigen, T-Cell, gamma-delta
  • fas Receptor
  • Cyclosporine