Rademaker M, Thomas R H, Kirby J D, Kovacs I B
Department of Dermatology, St. Bartholomew's Hospital, London, U.K.
Clin Exp Rheumatol. 1991 May-Jun;9(3):247-51.
The rheology of red blood cells in patients with systemic sclerosis is abnormal. To investigate this further we have examined the effect of sera from patients with systemic sclerosis on the handling of calcium ions by the erythrocyte membrane. Normal erythrocytes were filled with a photoprotein (aequorin) which emits light on contact with calcium. These photoprotein loaded normal erythrocytes were then incubated overnight with serum from: normal subjects (n = 20), from patients with systemic sclerosis (n = 27) or from patients with primary Raynaud's disease (n = 10). There was no significant difference in basal calcium leakage, as measured by the amount of light produced following the addition of triton X-100. Induced calcium influx, as measured by the amount of light produced following the addition of ionophore A23817, was significantly greater in the photoprotein loaded erythrocytes incubated overnight with serum from patients with systemic sclerosis compared to those incubated with serum from normal subjects (p less than 0.02) or patients with primary Raynaud's disease (p less than 0.01). This modulation of Ca2+ handling in erythrocytes by a serum factor from patients with systemic sclerosis could account for the alterations in erythrocyte function, such as red cell deformability, observed in systemic sclerosis.
系统性硬化症患者红细胞的流变学异常。为进一步研究这一现象,我们检测了系统性硬化症患者血清对红细胞膜处理钙离子的影响。正常红细胞内填充有光蛋白(水母发光蛋白),该蛋白与钙接触时会发光。然后,将这些装载了光蛋白的正常红细胞与以下血清孵育过夜:正常受试者(n = 20)、系统性硬化症患者(n = 27)或原发性雷诺病患者(n = 10)。通过添加曲拉通X - 100后产生的光量来测量基础钙泄漏,结果无显著差异。通过添加离子载体A23817后产生的光量来测量诱导性钙内流,与用正常受试者血清(p < 0.02)或原发性雷诺病患者血清(p < 0.01)孵育过夜的装载了光蛋白的红细胞相比,用系统性硬化症患者血清孵育过夜的红细胞诱导性钙内流显著增加。系统性硬化症患者血清因子对红细胞钙处理的这种调节作用,可能解释了在系统性硬化症中观察到的红细胞功能改变,如红细胞变形性。