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大颗粒淋巴细胞白血病作为类风湿关节炎的一种并发症。

Large granular lymphocyte leukemia as a complication of rheumatoid arthritis.

作者信息

Lois Iglesias Ana, Sifuentes Giraldo Walter Alberto, Bachiller Corral Javier, Saiz González Ana, Brito Brito Elia, Zea Mendoza Antonio

机构信息

Servicio de Reumatología, Hospital Universitario Ramón y Cajal, Madrid, España.

出版信息

Reumatol Clin. 2012 Nov-Dec;8(6):365-7. doi: 10.1016/j.reuma.2011.12.014. Epub 2012 Apr 6.

Abstract

Large granular lymphocyte leukemia is a rare entity belonging to same spectrum of diseases than Felty's syndrome, which might occur in patients with long-standing rheumatoid arthritis. It is clinically characterized by persistent neutropenia and recurrent bacterial infections associated with the presence in both peripheral blood and bone marrow of clonal expansion of atypic lymphocytes with a cytotoxic T cell phenotype, or less frequently an NK-cell phenotype, as well as splenomegaly. It is more frequently diagnosed in seropositive rheumatoid arthritis, with significant structural damage, extra-articular manifestations and persistently elevated values of ESR, despite them havubg low inflammatory joint activity. We report the case of a 70 year old male with a long-standing rheumatoid arthritis, who developed septic shock secondary to prosthetic hip infection by Salmonella spp. He showed persistent neutropenia, and an aberrant monoclonal T cell population was detected in both peripheral blood and bone marrow, consistent with large granular lymphocyte leukemia.

摘要

大颗粒淋巴细胞白血病是一种罕见的疾病,与费尔蒂综合征属于同一疾病谱,后者可能发生在长期类风湿关节炎患者中。其临床特征为持续性中性粒细胞减少和反复细菌感染,外周血和骨髓中存在具有细胞毒性T细胞表型(或较少见的NK细胞表型)的非典型淋巴细胞克隆性扩增,以及脾肿大。它在血清学阳性的类风湿关节炎中更常被诊断出来,尽管炎症性关节活动度较低,但仍有明显的结构损伤、关节外表现和血沉持续升高。我们报告一例70岁男性,患有长期类风湿关节炎,因沙门氏菌属导致人工髋关节感染继发感染性休克。他表现出持续性中性粒细胞减少,在外周血和骨髓中均检测到异常的单克隆T细胞群,符合大颗粒淋巴细胞白血病。

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