title: 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.
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.
authors.affiliation: Third Department of Internal Medicine, Gunma University School of Medicine, Maebashi, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Saitoh
authors.forename: M
authors.initials: M
authors.lastname: Karasawa
authors.forename: M
authors.initials: M
authors.lastname: Sakuraya
authors.forename: N
authors.initials: N
authors.lastname: Norio
authors.forename: T
authors.initials: T
authors.lastname: Junko
authors.forename: K
authors.initials: K
authors.lastname: Shirakawa
authors.forename: T
authors.initials: T
authors.lastname: Matsushima
authors.forename: N
authors.initials: N
authors.lastname: Tsukamoto
authors.forename: Y
authors.initials: Y
authors.lastname: Nojima
authors.forename: H
authors.initials: H
authors.lastname: Murakami
mesh_terms: D017252:CD3 Complex / Q000097:blood; D016572:Cyclosporine / Q000008:administration & dosage*; D006801:Humans; D016130:Immunophenotyping; D007945:Leukemia, Lymphoid / Q000150:complications / Q000188:drug therapy*; D015458:Leukemia, T-Cell / Q000150:complications / Q000188:drug therapy*; D008297:Male; D008875:Middle Aged; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000097:blood; D012010:Red-Cell Aplasia, Pure / Q000188:drug therapy / Q000209:etiology; D013922:Thrombocytosis / Q000188:drug therapy / Q000209:etiology; D016896:Treatment Outcome; D019014:fas Receptor / Q000097:blood
chemical_list: D017252:CD3 Complex; D016692:Receptors, Antigen, T-Cell, gamma-delta; D019014:fas Receptor; D016572:Cyclosporine