Icahn School of Medicine at Mount Sinai, New York, NY, USA.
NYU Winthrop Hospital, Mineola, NY, USA.
J Investig Med High Impact Case Rep. 2020 Jan-Dec;8:2324709620941303. doi: 10.1177/2324709620941303.
Large granular lymphocyte leukemia (LGL) is a clonal, lymphoproliferative disorder with an indolent disease course. T-cell LGL (T-LGL) is the most common type of LGL driven from T-cell lineage (85%). The coexistence of T-LGL with several types of autoimmune disorders, mostly rheumatoid arthritis (RA), has been reported. Felty's syndrome (FS) is defined by splenomegaly, low neutrophil count, and destructive arthritis and is usually seen in <1% of patients with RA. About 30% to 40% of patients with FS have been reported to have an expansion of large granulated lymphocytes in the circulation. FS and T-LGL are similar in terms of clinical manifestations, response to immunosuppressive therapy, their smoldering course, and immunogenetic findings, proposing FS and T-LGL with RA might be different aspects of a single disease spectrum. In this article, we present a case with long-standing RA who had never been on DMARD (Disease Modifying Anti-Rheumatic Drugs) treatment found to have constitutional symptoms, neutropenia, and splenomegaly, and the patient was diagnosed with T-LGL.
大颗粒淋巴细胞白血病(LGL)是一种惰性的克隆性淋巴增殖性疾病。T 细胞 LGL(T-LGL)是最常见的 LGL 类型,来源于 T 细胞谱系(85%)。已经报道了 T-LGL 与多种自身免疫性疾病共存,主要是类风湿关节炎(RA)。 Felty 综合征(FS)的定义是脾肿大、中性粒细胞计数降低和破坏性关节炎,通常见于 <1%的 RA 患者。约 30%至 40%的 FS 患者报告有循环中大颗粒淋巴细胞的扩张。FS 和 T-LGL 在临床表现、免疫抑制治疗反应、潜伏病程和免疫遗传学发现方面相似,提示 FS 和 T-LGL 伴 RA 可能是同一疾病谱的不同方面。在本文中,我们报告了一例长期患有 RA 的患者,从未接受过 DMARD(疾病修饰抗风湿药物)治疗,发现有全身症状、中性粒细胞减少和脾肿大,患者被诊断为 T-LGL。