title: Induction of clinical remission in T-large granular lymphocyte leukemia with cyclosporin A, monitored by use of immunophenotyping with Vbeta antibodies.
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.
authors.affiliation: Department of Internal Medicine, St Elisabeth Hospital, Tilburg, The Netherlands.
authors.forename: K
authors.initials: K
authors.lastname: Brinkman
authors.forename: J J
authors.initials: JJ
authors.lastname: van Dongen
authors.forename: K
authors.initials: K
authors.lastname: van Lom
authors.forename: K
authors.initials: K
authors.lastname: Groeneveld
authors.forename: J F
authors.initials: JF
authors.lastname: Miseré
authors.forename: C
authors.initials: C
authors.lastname: van der Heul
mesh_terms: D000753:Anemia, Refractory / Q000097:blood / Q000150:complications; D000912:Antibodies, Neoplasm / Q000032:analysis*; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D016572:Cyclosporine / Q000627:therapeutic use*; D005260:Female; D019674:Genes, T-Cell Receptor beta; D006801:Humans; D007135:Immunoglobulin Variable Region / Q000276:immunology*; D016130:Immunophenotyping; D007945:Leukemia, Lymphoid / Q000188:drug therapy* / Q000235:genetics / Q000276:immunology; D015458:Leukemia, T-Cell / Q000188:drug therapy* / Q000235:genetics / Q000276:immunology; D008875:Middle Aged; D009503:Neutropenia / Q000097:blood / Q000150:complications; D012074:Remission Induction; D013921:Thrombocytopenia / Q000097:blood / Q000150:complications
chemical_list: D000912:Antibodies, Neoplasm; D000970:Antineoplastic Agents; D007135:Immunoglobulin Variable Region; D016572:Cyclosporine