文章：

大颗粒淋巴细胞白血病：临床特征、分子发病机制、诊断与治疗

Large Granular Lymphocytic Leukemia: Clinical Features, Molecular Pathogenesis, Diagnosis and Treatment

原文发布日期：27 March 2024

DOI: 10.3390/cancers16071307

类型: Article

开放获取: 是

英文摘要：

Large granular lymphocytic (LGL) leukemia is a lymphoproliferative disorder characterized by persistent clonal expansion of mature T- or natural killer cells in the blood via chronic antigenic stimulation. LGL leukemia is associated with specific immunophenotypic and molecular features, particularlySTAT3andSTAT5mutations and activation of theJAK-STAT3, Fas/Fas-L andNF-κBsignaling pathways. Disease-related deaths are mainly due to recurrent infections linked to severe neutropenia. The current treatment is based on immunosuppressive therapies, which frequently produce unsatisfactory long-term responses, and for this reason, personalized approaches and targeted therapies are needed. Here, we discuss molecular pathogenesis, clinical presentation, associated autoimmune disorders, and the available treatment options, including emerging therapies.

摘要翻译： 

大颗粒淋巴细胞（LGL）白血病是一种淋巴增殖性疾病，其特征为成熟T细胞或自然杀伤细胞在慢性抗原刺激下于血液中持续克隆性扩增。该疾病与特定的免疫表型和分子特征相关，尤其是STAT3和STAT5基因突变以及JAK-STAT3、Fas/Fas-L和NF-κB信号通路的激活。疾病相关死亡主要归因于与严重中性粒细胞减少相关的反复感染。当前治疗以免疫抑制疗法为主，但其长期疗效常不理想，因此需要个体化方案及靶向治疗。本文讨论了LGL白血病的分子发病机制、临床表现、相关自身免疫性疾病以及现有治疗选择，包括新兴疗法。

原文链接：

Large Granular Lymphocytic Leukemia: Clinical Features, Molecular Pathogenesis, Diagnosis and Treatment