文章：

一项基于人群的大颗粒淋巴细胞白血病研究

A population-based study of large granular lymphocyte leukemia

原文发布日期：2016-08-05

DOI: 10.1038/bcj.2016.59

类型: Original Article

开放获取: 是

英文摘要：

Large granular lymphocyte (LGL) leukemia is a lymphoproliferative disorder of cytotoxic cells. T-cell LGL (T-LGL) leukemia is characterized by accumulation of cytotoxic T cells in blood and infiltration of the bone marrow, liver or spleen. Population-based studies have not been reported in LGL leukemia. We present clinical characteristics, natural history and risk factors for poor survival in patients with LGL leukemia using the Surveillance, Epidemiology, and End Results Program (SEER) and the United States National Cancer Data Base (NCDB). LGL leukemia is an extremely rare disease with the incidence of 0.2 cases per 1 000 000 individuals. The median age at diagnosis was 66.5 years with females likely to be diagnosed at 3 years earlier compared with males. Analysis of patient-level data using NCDB (n=978) showed that 45% patients with T-LGL leukemia required some form of systemic treatment at the time of diagnosis. T-LGL leukemia patients have reduced survival compared with general population, with a median overall survival of 9 years. Multivariate analysis showed that age >60 years at the time of diagnosis and the presence of significant comorbidities were independent predictors of poor survival.

摘要翻译： 

大颗粒淋巴细胞白血病是一种细胞毒性细胞的淋巴增殖性疾病。T细胞大颗粒淋巴细胞白血病的特点是细胞毒性T细胞在血液中积累，并浸润骨髓、肝脏或脾脏。目前尚未有基于人群的大颗粒淋巴细胞白血病研究报告。我们利用监测、流行病学和最终结果数据库及美国国家癌症数据库，展示了大颗粒淋巴细胞白血病患者的临床特征、自然史以及不良生存的风险因素。大颗粒淋巴细胞白血病是一种极为罕见的疾病，发病率为每百万人0.2例。诊断时的中位年龄为66.5岁，女性比男性早3年被诊断出来。使用国家癌症数据库对患者个体数据进行的分析显示，45%的T细胞大颗粒淋巴细胞白血病患者在诊断时需要某种形式的全身治疗。与普通人群相比，T细胞大颗粒淋巴细胞白血病患者的生存期缩短，中位总生存期为9年。多变量分析表明，诊断时年龄大于60岁以及存在显著合并症是生存不良的独立预测因素。

原文链接：

A population-based study of large granular lymphocyte leukemia