Bowman S J, Bhavnani M, Geddes G C, Corrigall V, Boylston A W, Panayi G S, Lanchbury J S
Division of Medicine, UMDS, Guy's Hospital, London, UK.
Clin Exp Immunol. 1995 Jul;101(1):18-24. doi: 10.1111/j.1365-2249.1995.tb02271.x.
Felty's syndrome (FS), the association of rheumatoid arthritis (RA) and idiopathic neutropenia, remains an unexplained phenomenon. HLA-DR4 is found in over 90% of cases. Patients with FS may have a T cell lymphocytosis of CD3+CD8+CD57+ large granular lymphocytes (LGL syndrome). In this study of 47 patients with FS, 19% had clear evidence for LGL expansions, while in total 42% had variable evidence for the LGL syndrome using currently available techniques. Of these T cell expansions, 76% were clonal, as demonstrated by Southern blotting and analysis with T cell receptor (TCR) beta chain constant region probes. This technique may fail to detect clonal populations in some patients. Cytofluorographic analysis using antibodies specific for TCR V beta chains identified patients with clonal LGL expansions with results comparable to those obtained with Southern blotting. No evidence for shared V beta usage among expansions from different patients was seen. The role of LGL in RA and FS is currently unclear, but this technique offers a practical and accessible means of identifying patients with LGL expansions, as a starting point for further investigation.
费尔蒂综合征（FS），即类风湿关节炎（RA）与特发性中性粒细胞减少症的关联，仍是一种无法解释的现象。超过90%的病例中可发现HLA - DR4。FS患者可能存在CD3 + CD8 + CD57 +大颗粒淋巴细胞的T细胞淋巴细胞增多症（LGL综合征）。在这项对47例FS患者的研究中，19%有明确的LGL扩增证据，而使用现有技术总体上42%有LGL综合征的可变证据。在这些T细胞扩增中，76%是克隆性的，这通过Southern印迹法以及用T细胞受体（TCR）β链恒定区探针进行分析得以证明。该技术可能无法检测出某些患者中的克隆群体。使用针对TCR Vβ链的特异性抗体进行细胞荧光分析可识别出具有克隆性LGL扩增的患者，其结果与Southern印迹法所得结果相当。未发现不同患者的扩增之间存在共享Vβ使用的证据。LGL在RA和FS中的作用目前尚不清楚，但该技术提供了一种实用且可及的方法来识别具有LGL扩增的患者，作为进一步研究的起点。
