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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

2000; Wiley; Volume: 65; Issue: 4 Linguagem: Inglês

10.1034/j.1600-0609.2000.065004272.x

1600-0609

Takayuki Saitoh, Masamitsu Karasawa, Masataka Sakuraya, Nakamura Norio, Junko Tohma, Kamon Shirakawa, Takafumi Matsushima, Norifumi Tsukamoto, Jun‐ichi Tamura, Takuji Naruse, Hirokazu Murakami,

Immune Cell Function and Interaction

Abstract: We report a case of γδ T‐cell‐type large granular lymphocyte (LGL) leukemia (CD3+,CD8+, CD57+,TCR γδ+), which was accompanied by pure red cell aplasia, neutropenia and thrombocytosis. Southern blotting analysis of the T‐cell receptor β gene showed the germline configuration, but clonal TCR J γ 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‐α), interferon gamma (IFN‐γ), interleukin‐1 beta (IL‐1β), 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.