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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
Author(s) -
Saitoh Takayuki,
Karasawa Masamitsu,
Sakuraya Masataka,
Norio Nakamura,
Junko Tohma,
Shirakawa Kamon,
Matsushima Takafumi,
Tsukamoto Norifumi,
Tamura Jun'ichi,
Naruse Takuji,
Murakami Hirokazu
Publication year - 2000
Publication title -
european journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0902-4441
DOI - 10.1034/j.1600-0609.2000.065004272.x
Subject(s) - fas ligand , immunology , cd8 , pure red cell aplasia , leukemia , cd3 , thrombopoietin , lymphocyte , tumor necrosis factor alpha , t cell receptor , thrombocytosis , biology , medicine , t cell , haematopoiesis , antigen , apoptosis , immune system , bone marrow , programmed cell death , platelet , biochemistry , genetics , stem cell
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.