Starkebaum G, Loughran T P, Gaur L K, Davis P, Nepom B S
VA Medical Center and University of Washington, Seattle, USA.
Arthritis Rheum. 1997 Apr;40(4):624-6. doi: 10.1002/art.1780400406.
Patients with chronic clonal proliferation of large granular lymphocytes (LGL leukemia) often have splenomegaly, neutropenia, and rheumatoid arthritis (RA), thereby resembling the manifestations observed in patients with Felty's syndrome. The present study sought to determine whether patients with these disorders represent 2 distinct subsets of neutropenic RA.
Prospective cohort study of outpatients attending clinics in university and private hospitals and in offices of private practice physicians. Twenty-two patients with Felty's syndrome and 22 patients with LGL leukemia, 10 of whom had RA, were studied. HLA genotyping was performed on peripheral blood mononuclear leukocyte genomic DNA.
Nineteen of the 22 patients with Felty's syndrome (86%) were DR4 positive. Nine of the 10 patients with LGL leukemia plus RA were also DR4 positive. In contrast, only 4 of the 12 patients with LGL leukemia without RA (33%) were DR4 positive, a frequency that was within the normal range.
The finding of an equally high prevalence of DR4 in patients with Felty's syndrome and in those with LGL leukemia plus RA suggests that both disorders have a similar immunogenetic basis and are parts of a single disease process rather than 2 separate disorders.
大颗粒淋巴细胞慢性克隆性增殖患者（大颗粒淋巴细胞白血病）常出现脾肿大、中性粒细胞减少和类风湿关节炎（RA），临床表现与费尔蒂综合征患者相似。本研究旨在确定这些疾病患者是否代表中性粒细胞减少型类风湿关节炎的两个不同亚组。
对在大学医院、私立医院门诊以及私人执业医生办公室就诊的门诊患者进行前瞻性队列研究。研究了22例费尔蒂综合征患者和22例大颗粒淋巴细胞白血病患者，其中10例大颗粒淋巴细胞白血病患者患有类风湿关节炎。对外周血单个核白细胞基因组DNA进行HLA基因分型。
22例费尔蒂综合征患者中有19例（86%）DR4呈阳性。10例大颗粒淋巴细胞白血病合并类风湿关节炎患者中有9例DR4也呈阳性。相比之下，12例无类风湿关节炎的大颗粒淋巴细胞白血病患者中只有4例（33%）DR4呈阳性，该频率在正常范围内。
费尔蒂综合征患者与大颗粒淋巴细胞白血病合并类风湿关节炎患者中DR4的高患病率相同，这表明这两种疾病具有相似的免疫遗传基础，是单一疾病过程的一部分，而非两种独立的疾病。
