Department of Medicine, Penn State Hershey Cancer Institute, Pennsylvania State University College of Medicine, Hershey, Pennsylvania 17033-0850, USA.
Curr Opin Hematol. 2011 Jul;18(4):254-9. doi: 10.1097/MOH.0b013e32834760fb.
Patients with chronic large granular lymphocyte (LGL) leukemia often have rheumatoid arthritis (RA), neutropenia and splenomegaly, thereby resembling the manifestations observed in patients with Felty's syndrome, which is a rare complication of RA characterized by neutropenia and splenomegaly. Both entities have similar clinical and laboratory presentation, as well as a common genetic determinant, HLA-DR4, indicating they may be part of the same disease spectrum. This review paper seeks to discuss the underlying pathogenesis and therapeutic algorithm of RA, neutropenia and splenomegaly in the spectrum of LGL leukemia and Felty's syndrome.
We hypothesize that there may be a common pathogenic mechanism between LGL leukemia and typical Felty's syndrome. Phenotypic and functional data have strongly suggested that CD3 LGL leukemia is antigen-activated. Aberrations in the T-cell repertoire with the emergence of oligoclonal/clonal lymphoid populations have been found to play a pivotal role in pathogenesis of RA. The biologic properties of the pivotal T cell involved in RA pathogenesis are remarkably similar to those in leukemic LGL.
RA-associated T-cell LGL leukemia and articular manifestations of typical Felty's syndrome are not distinguishable. A common pathogenetic link between LGL leukemia and RA is proposed.
慢性大颗粒淋巴细胞（LGL）白血病患者常伴有类风湿关节炎（RA）、中性粒细胞减少和脾肿大，类似于 Felty 综合征的表现，后者是 RA 的一种罕见并发症，其特征为中性粒细胞减少和脾肿大。这两种疾病具有相似的临床表现和实验室表现，以及共同的遗传决定因素 HLA-DR4，表明它们可能属于同一疾病谱。本文旨在讨论 LGL 白血病和 Felty 综合征谱系中 RA、中性粒细胞减少和脾肿大的潜在发病机制和治疗方案。
我们假设 LGL 白血病和典型 Felty 综合征之间可能存在共同的发病机制。表型和功能数据强烈表明，CD3+LGL 白血病是抗原激活的。T 细胞库的异常，出现寡克隆/克隆性淋巴细胞群，在 RA 的发病机制中起着关键作用。涉及 RA 发病机制的关键 T 细胞的生物学特性与白血病性 LGL 非常相似。
与 RA 相关的 T 细胞 LGL 白血病和典型 Felty 综合征的关节表现难以区分。提出了 LGL 白血病和 RA 之间存在共同的发病机制。
