Induction of clinical remission in T-large granular lymphocyte leukemia with cyclosporin A, monitored by use of immunophenotyping with Vbeta antibodies

Leukemia. 1998 Feb;12(2):150-4. doi: 10.1038/sj.leu.2400907.

Abstract

A 54-year-old woman presented with a severe autoimmune anemia, thrombocytopenia, neutropenia (Evans' syndrome), and CD8+ lymphocytosis, without signs of lymphadenopathy or splenomegaly. A diagnosis of T cell large granular lymphocyte (T-LGL) leukemia was made, based on cytomorphology, the typical CD3+/CD4-/CD8+/CD16+/CD56-/CD57-/HLA-DR(+/-) immunophenotype of the lymphocytosis (9 x 10(9)/l), and biallelic clonally rearranged T cell receptor beta (TCR beta) genes. Clonality of the TCR alphabeta+ T-LGL was also demonstrated with a panel of antibodies against variable domains of TCR beta chains, which showed single Vbeta7.1 expression on the CD3+ T-lymphocytes. After treatment failure with corticosteroids, splenectomy, and cyclophosphamide, respectively, a complete clinical remission was induced and sustained with cyclosporin A. Vbeta7.1/CD8/CD3 triple immunofluorescence stainings appeared to be valuable for titrating the cyclosporin A dosage by monitoring the T-LGL cells during treatment.

Publication types

  • Case Reports

MeSH terms

  • Anemia, Refractory / blood
  • Anemia, Refractory / complications
  • Antibodies, Neoplasm / analysis*
  • Antineoplastic Agents / therapeutic use*
  • Cyclosporine / therapeutic use*
  • Female
  • Genes, T-Cell Receptor beta
  • Humans
  • Immunoglobulin Variable Region / immunology*
  • Immunophenotyping
  • Leukemia, Lymphoid / drug therapy*
  • Leukemia, Lymphoid / genetics
  • Leukemia, Lymphoid / immunology
  • Leukemia, T-Cell / drug therapy*
  • Leukemia, T-Cell / genetics
  • Leukemia, T-Cell / immunology
  • Middle Aged
  • Neutropenia / blood
  • Neutropenia / complications
  • Remission Induction
  • Thrombocytopenia / blood
  • Thrombocytopenia / complications

Substances

  • Antibodies, Neoplasm
  • Antineoplastic Agents
  • Immunoglobulin Variable Region
  • Cyclosporine