Moosig F, Schoch R, Kneba M
Medizinische Klinik des Universitätsklinikum Schleswig-Holstein, Campus Kiel.
Z Rheumatol. 2006 Sep;65(5):447-51. doi: 10.1007/s00393-005-0013-4.
T-Large Granular Lymphocyte (T-LGL) leukaemia is a rare clonal disease characterized by neutropenia and/or anaemia. Because of its strong association with rheumatoid arthritis (RA), T-LGL leukaemia is an important differential diagnosis to Felty's syndrome. This differentiation might be especially difficult since, in severe RA with extraarticular manifestations, there is often an expanded memory effector T-cell population which can hardly be separated from T-LGL leukaemia cells by means of immunophenotyping. The main criterion for T-LGL leukaemia is the detection of a clonal T-cell-receptor rearrangement by PCR. First-line therapy consists of weekly low-dose methotrexate. Alternatively, other immunosuppressives or cytotoxic agents can be useful. There are very limited data from therapy studies. The German CLL study group has initiated a protocol using parenteral low-dose methotrexate as first-line therapy and fludarabine as second-line medication.
T 大颗粒淋巴细胞（T-LGL）白血病是一种罕见的克隆性疾病，其特征为中性粒细胞减少和/或贫血。由于它与类风湿性关节炎（RA）密切相关，T-LGL 白血病是费尔蒂综合征的重要鉴别诊断。这种鉴别可能特别困难，因为在伴有关节外表现的重度 RA 中，通常存在记忆效应 T 细胞群体扩大的情况，通过免疫表型分析几乎无法将其与 T-LGL 白血病细胞区分开来。T-LGL 白血病的主要诊断标准是通过聚合酶链反应（PCR）检测克隆性 T 细胞受体重排。一线治疗包括每周一次的小剂量甲氨蝶呤。另外，其他免疫抑制剂或细胞毒性药物可能也有效。治疗研究的数据非常有限。德国慢性淋巴细胞白血病研究组已启动一项方案，使用胃肠外小剂量甲氨蝶呤作为一线治疗，氟达拉滨作为二线用药。
