大型颗粒淋巴细胞白血病（LGLL）是一种慢性淋巴增生性疾病，其特征是T或NK细胞在外周血、脾和骨髓中的增殖。中性粒细胞减少导致反复感染是LGLL的主要表现。LGLL的一个特殊之处在于其经常与自身免疫性疾病相关联，其中最重要的是类风湿性关节炎，以及其他血液病，包括纯红细胞再生障碍和骨髓衰竭。多种表现和典型的亚急性病程有助于诊断困难和低诊断率的频繁出现。重要的是，免疫失调表现会随着LGLL的治疗而消失，因为血细胞计数正常化，这为两个实体之间的病理联系提供了有力的论据。治疗挑战在于第一线免疫抑制药物治疗后高复发率。新的靶向药物，其中一些目前已获批用于自身免疫性疾病，似乎是治疗LGLL的相关治疗策略，通过靶向参与疾病发病机制的关键激活途径，包括JAK-STAT信号通路。版权所有 © 2023 Elsevier Masson SAS出版。

Large granular lymphocyte leukemia (LGLL) is a chronic lymphoproliferative disorder characterized by the proliferation of T or NK cytotoxic cells in the peripheral blood, the spleen and the bone marrow. Neutropenia leading to recurrent infections represents the main manifestation of LGLL. One specificity of LGLL is its frequent association with auto-immune disorders, among them first and foremost rheumatoid arthritis, and other hematologic diseases, including pure red cell aplasia and bone marrow failure. The large spectrum of manifestations and the classical indolent course contribute to the diagnosis difficulties and the frequency of underdiagnosed cases. Of importance, the dysimmune manifestations disappear with the treatment of LGLL as the blood cell counts normalize, giving a strong argument for a pathological link between the two entities. The therapeutic challenge results from the high rate of relapses following the first line of immunosuppressive drugs. New targeted agents, some of which are currently approved in autoimmune diseases, appear to be relevant therapeutic strategies to treat LGLL, by targeting key activated pathways involved in the pathogenesis of the disease, including JAK-STAT signaling.Copyright © 2023. Published by Elsevier Masson SAS.