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 109/l), and biallelic clonally rearranged T cell receptor β (TCRβ) genes. Clonality of the TCRαβ+ T-LGL was also demonstrated with a panel of antibodies against variable domains of TCRβ chains, which showed single Vβ7.1 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. Vβ7.1/CD8/CD3 triple immunofluorescence stainings appeared to be valuable for titrating the cyclosporin A dosage by monitoring the T-LGL cells during treatment.

Cyclosporin A, Evans' syndrome, Leukemia, T-LGL leukemia, TCR genes, Vβ antibodies
dx.doi.org/10.1038/sj.leu.2400907, hdl.handle.net/1765/68031
Leukemia
Department of Immunology

Brinkman, K, van Dongen, J.J.M, van Lom, K, Groeneveld, K, Miseré, J.F.M.M, & van der Heul, C. (1998). Induction of clinical remission in T-large granular lymphocyte leukemia with cyclosporin A, monitored by use of immunophenotyping with Vβ antibodies. Leukemia, 12(2), 150–154. doi:10.1038/sj.leu.2400907