• 主题
高级搜索  >
历史搜索 清空历史
首页> 外文期刊>Clinical immunology: The official journal of the Clinical Immunology Society >T cell large granular lymphocyte leukemia associated with rheumatoid arthritis and neutropenia.
【24h】

T cell large granular lymphocyte leukemia associated with rheumatoid arthritis and neutropenia.

机译：T细胞大颗粒淋巴细胞白血病伴有类风湿关节炎和中性粒细胞减少症。

获取原文
获取原文并翻译 | 示例
           
页面导航
  • 摘要
  • 著录项
  • 相似文献
  • 相关主题

摘要

T cell large granular lymphocyte leukemia (T-LGL) is a disease characterized by clonal expansion of cytotoxic T cells (CTLs). It generally follows an indolent course and is notable for an association with chronic inflammation, neutropenia and rheumatoid arthritis (RA). We present herein a case of a patient with rheumatoid arthritis (RA), neutropenia, large granular lymphocytosis, and an expanded clonal population of peripheral blood CD3(+)CD8(+)TCRalphabeta CTLs, consistent with the diagnosis of T-LGL. T-LGL is part of a spectrum of large granular lymphocytic (LGL) disorders, which includes the more common indolent variety of this disease (as illustrated by the case herein), an aggressive but rare form of this leukemia, natural killer (NK) cell LGL leukemia, Felty's syndrome (FS), and chronic large granular lymphocytosis. T-LGL appears to be a relatively rare disease, but the true prevalence is not known. FS occurs in less than 1% of patients with RA and is typically defined by the triad of destructive arthritis, neutropenia, and variable splenomegaly. A subset of patients with FS will demonstrate polyclonal expansion of LGLs, implying a relationship between proliferation of LGLs and the mechanisms of neutropenia. Thus, T-LGL leukemia and FS with LGL expansion in the setting of RA is classically distinguished by the clonality of the CTL population, with monoclonality in T-LGL and polyclonality in FS. Despite this difference, T-LGL and FS are often similar in their clinical and biological behavior. Both may respond to immunosuppressive therapy, and pursue a smoldering course typical of a chronic inflammatory disease.
机译：T细胞大颗粒淋巴细胞白血病（T-LGL）是一种以细胞毒性T细胞（CTL）克隆扩增为特征的疾病。它通常伴随着缓慢的过程，并且与慢性炎症，中性粒细胞减少和类风湿关节炎（RA）有关。我们在本文中介绍了类风湿关节炎（RA），中性粒细胞减少，大颗粒淋巴细胞增多以及周围血CD3（+）CD8（+）TCRalphabeta CTL克隆人群扩大的病例，与T-LGL的诊断一致。 T-LGL是大颗粒淋巴细胞（LGL）疾病谱系的一部分，其中包括该疾病的更常见的惰性疾病（如此处的案例所示），这是这种白血病的一种侵袭性但罕见的自然杀手（NK）细胞LGL白血病，Felty综合征（FS）和慢性大颗粒淋巴细胞增多。 T-LGL似乎是一种相对罕见的疾病，但真正的患病率尚不清楚。 FS发生在少于1％的RA患者中，通常由破坏性关节炎，中性粒细胞减少和可变性脾肿大的三联征定义。 FS患者的一部分将表现出LGL的多克隆扩增，这暗示LGL的增殖与中性粒细胞减少的机制之间的关系。因此，RA背景下T-LGL白血病和FS伴随LGL扩展的特点是CTL人群的克隆性，在T-LGL中具有单克隆性，在FS中具有多克隆性。尽管存在这种差异，但T-LGL和FS的临床和生物学行为通常相似。两者都可能对免疫抑制疗法有反应，并且会经历慢性炎症性疾病典型的阴燃过程。

著录项

相似文献

  • 外文文献
  • 中文文献
  • 专利
获取原文

客服邮箱：kefu@zhangqiaokeyan.com

京公网安备：11010802029741号 ICP备案号：京ICP备15016152号-6 六维联合信息科技 (北京) 有限公司©版权所有

  • 客服微信

  • 服务号