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[费尔蒂综合征]

[Felty's syndrome].

作者信息

Larsen M R, May O

机构信息

Odense Sygehus, geriatrisk afdeling Q.

出版信息

Ugeskr Laeger. 1991 Jul 8;153(28):1990-3.

PMID:1862580
Abstract

Felty's syndrome (FS) consists of the triad: rheumatoid arthritis (RA), leukopenia and splenomegaly. FS occurs in approximately 1% of patients with RA. In this syndrome, the risk of infection is increased and anaemia, thrombocytopenia and cutaneous ulcers are more frequently observed. The literature is reviewed on the basis of a case history. The pathogenesis is unknown but is probably multifactorial. Cell antibodies, increased occurrence of immune complexes, inhibited neutrophil production, altered neutrophil distribution and reduced neutrophil function have been observed. The main indication for treatment is present if the patient has severe neutropenia (less than 0.1 x 10(9)/l) and repeated infections. Various methods of treatment are available. The most important are: gold, low-dose methotrexate, lithium, methylprednisolone pulse therapy, penicillamine and splenectomy. According to the literature, conventional steroid treatment cannot be recommended.

摘要

费尔蒂综合征(FS)由类风湿关节炎(RA)、白细胞减少症和脾肿大三联征组成。FS约见于1%的RA患者。在此综合征中,感染风险增加,贫血、血小板减少症和皮肤溃疡更为常见。本文基于一例病史对相关文献进行综述。其发病机制尚不清楚,但可能是多因素的。已观察到细胞抗体、免疫复合物发生率增加、中性粒细胞生成受抑制、中性粒细胞分布改变以及中性粒细胞功能降低。如果患者出现严重中性粒细胞减少症(低于0.1×10⁹/L)且反复感染,则存在主要治疗指征。有多种治疗方法可供选择。最重要的有:金制剂、小剂量甲氨蝶呤、锂盐、甲泼尼龙冲击疗法、青霉胺和脾切除术。根据文献,不推荐常规类固醇治疗。

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