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

A 54-year-old woman presented with a severe autoimmune anemia, thrombacytapenia, neutropenia (Evans' syndrome), and CD8(+) lymphocytosis, without signs of lymphradenopathy 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)/I), and biallelic clonally rearranged T cell receptor beta (TCR beta) genes. Clonality of the TGR alpha beta(+) T-LGL was also demonstrated with a panel of antibodies against variable domains of TCR beta chains, which showed single V beta 7.1 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. V beta 7.CD8/CD3 triple immunofluorescence stainings appeared to he valuable for titrating the cyclosporin A dosage by monitoring the T-LGL cells during treatment.