Loughran T P, Starkebaum G, Kidd P, Neiman P
Department of Medicine, University of Washington School of Medicine, Seattle.
Arthritis Rheum. 1988 Jan;31(1):31-6. doi: 10.1002/art.1780310105.
Some patients with chronically elevated large granular lymphocyte (LGL) numbers have rheumatoid arthritis (RA). Since these patients also may have neutropenia and splenomegaly, their symptoms resemble those of patients diagnosed as having Felty's syndrome (FS). We studied the immunophenotypic and genotypic characteristics of mononuclear cells from patients with RA and neutropenia to better determine the extent of heterogeneity in this condition. Four patients had markedly increased numbers of LGLs, which expressed HNK-1 antigen and IgG Fc receptors. In contrast, the remaining 8 patients, who had FS, had normal LGL counts, and surface marker studies showed normal numbers of HNK-1 and IgG Fc receptor positive cells. Clonal rearrangement of the T cell receptor beta chain gene was demonstrated in all 4 patients with excess LGLs, whereas a germline configuration of this gene was present in all 6 FS patients in whom this was studied. These results suggest that there are diverse groups among patients with RA and neutropenia. Since prognosis may differ, it is important to recognize that some patients who are considered to have Felty's syndrome may have a clonal proliferation of LGLs.
一些大颗粒淋巴细胞（LGL）数量长期升高的患者患有类风湿性关节炎（RA）。由于这些患者也可能有中性粒细胞减少和脾肿大，他们的症状与被诊断为费尔蒂综合征（FS）的患者相似。我们研究了RA伴中性粒细胞减少患者单核细胞的免疫表型和基因型特征，以更好地确定这种情况下的异质性程度。4例患者的LGL数量显著增加，这些LGL表达HNK-1抗原和IgG Fc受体。相比之下，其余8例患有FS的患者LGL计数正常，表面标志物研究显示HNK-1和IgG Fc受体阳性细胞数量正常。在所有4例LGL过多的患者中均证实了T细胞受体β链基因的克隆重排，而在所有6例接受此项研究的FS患者中该基因呈种系构型。这些结果表明，RA伴中性粒细胞减少的患者存在不同的群体。由于预后可能不同，认识到一些被认为患有费尔蒂综合征的患者可能存在LGL的克隆增殖很重要。
