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应用造血生长因子粒细胞集落刺激因子(G-CSF)治疗费尔蒂综合征。

Treatment of Felty's syndrome with the haemopoietic growth factor granulocyte colony-stimulating factor (G-CSF).

作者信息

Stanworth S J, Bhavnani M, Chattopadhya C, Miller H, Swinson D R

机构信息

Department of Haematology, Manchester Royal Infirmary.

出版信息

QJM. 1998 Jan;91(1):49-56. doi: 10.1093/qjmed/91.1.49.

Abstract

Felty's syndrome (FS) (rheumatoid arthritis with neutropenia and splenomegaly) has a poor prognosis, largely because of the high risk of severe infection. Granulocyte colony-stimulating factor (G-CSF) is an emerging treatment for chronic neutropenia. We prospectively monitored its use in eight patients with recurrent infections or who required joint surgery. Significant side-effects were documented in five, including nausea, malaise, generalized joint pains, and in one patient, a vasculitic skin rash. In two patients treatment had to be stopped, and in these cases G-CSF had been started at full vial dosage (300 micrograms/ml filgrastim or 263 micrograms/ml lenograstim) alternate days or daily. G-CSF treatment was continued in three patients by restarting at reduced dose, and changing the proprietary formulation. G-CSF raised the neutrophil count, reduced severe infection, and allowed surgery to be performed. A combined clinical and laboratory index suggested that long-term treatment (up to 3.5 years) did not exacerbate the arthritis. Once on established treatment, it may be possible to use smaller weekly doses of G-CSF to maintain the same clinical benefit. One of the three patients whose FS was associated with a large granular T-cell lymphocytosis showed a reduction in this subset of lymphocytes during G-CSF treatment.

摘要

费尔蒂综合征(FS)(类风湿关节炎伴中性粒细胞减少和脾肿大)预后较差,主要原因是严重感染风险高。粒细胞集落刺激因子(G-CSF)是一种用于治疗慢性中性粒细胞减少症的新兴疗法。我们前瞻性地监测了其在8例反复感染或需要进行关节手术的患者中的使用情况。记录到5例出现显著副作用,包括恶心、不适、全身关节疼痛,1例出现血管炎性皮疹。2例患者不得不停止治疗,在这些病例中,G-CSF开始以全瓶剂量(300微克/毫升非格司亭或263微克/毫升来格司亭)隔日或每日给药。3例患者通过降低剂量重新开始并更换专利制剂继续接受G-CSF治疗。G-CSF提高了中性粒细胞计数,减少了严重感染,并使手术得以进行。一项综合临床和实验室指标表明,长期治疗(长达3.5年)并未加重关节炎。一旦确立治疗方案,可能可以使用较小的每周剂量的G-CSF来维持相同的临床疗效。3例FS与大颗粒T细胞淋巴细胞增多相关的患者中,有1例在G-CSF治疗期间该淋巴细胞亚群减少。

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