Adams P C, Flanagan P R, Chau L, White M, Lazarovits A
Department of Medicine, University Hospital, University of Western Ontario, London.
Clin Invest Med. 1991 Oct;14(5):402-8.
To further evaluate a possible abnormality in the reticuloendothelial cells in hemochromatosis, the binding of a monoclonal anti-human liver ferritin antibody to monocytes was studied in 19 patients with hemochromatosis, 8 patients with secondary iron overload, 1 patient with hyperferritinemia without iron overload, and 15 normal volunteers. Binding of the antibody to the monocytes was analyzed using a fluorescence-activated cell sorter (FACS). Binding of the anti-ferritin antibody to monocytes was demonstrated in 34.7 +/- 4.5% (mean +/- standard error) of the monocytes in untreated hemochromatosis patients (mean serum ferritin = 2294 +/- 415 micrograms/L), 6.75 +/- 2.03% in treated hemochromatosis patients (mean serum ferritin = 263 +/- 85 micrograms/L), 12.3 +/- 2.7% of the monocytes in the secondary iron overload patients (mean serum ferritin = 2476 +/- 867 micrograms/L), 4.1% in the patient with hyperferritinemia (serum ferritin = 1192) and 4.1 +/- 0.5% of the monocytes in the normal volunteers (mean serum ferritin = 55.2 +/- 11.9 micrograms/L). % binding of anti-ferritin antibody was significantly greater in hemochromatosis patients compared to patients with secondary iron overload (p less than 0.05) despite a comparable degree of iron overload in the secondary iron overload group. The addition of exogenous human ferritin to samples from treated hemochromatosis patients and normal volunteers did not significantly increase the % of monocytes binding anti-ferritin antibody. These results suggest that monocytes from iron-loaded hemochromatosis patients express increased surface ferritin which may represent release of ferritin and a metabolic defect characteristic of hemochromatosis.
为了进一步评估血色素沉着症患者网状内皮细胞中可能存在的异常情况,我们对19例血色素沉着症患者、8例继发性铁过载患者、1例无铁过载的高铁蛋白血症患者以及15名正常志愿者进行了研究,观察单克隆抗人肝铁蛋白抗体与单核细胞的结合情况。使用荧光激活细胞分选仪(FACS)分析抗体与单核细胞的结合情况。未治疗的血色素沉着症患者(血清铁蛋白均值 = 2294 ± 415微克/升)中,34.7 ± 4.5%(均值 ± 标准误)的单核细胞显示有抗铁蛋白抗体结合;接受治疗的血色素沉着症患者(血清铁蛋白均值 = 263 ± 85微克/升)中,该比例为6.75 ± 2.03%;继发性铁过载患者(血清铁蛋白均值 = 2476 ± 867微克/升)中,12.3 ± 2.7%的单核细胞有抗体结合;高铁蛋白血症患者(血清铁蛋白 = 1192)中该比例为4.1%;正常志愿者(血清铁蛋白均值 = 55.2 ± 11.9微克/升)中,4.1 ± 0.5%的单核细胞有抗体结合。尽管继发性铁过载组的铁过载程度相当,但血色素沉着症患者抗铁蛋白抗体的结合百分比显著高于继发性铁过载患者(p < 0.05)。向接受治疗的血色素沉着症患者和正常志愿者的样本中添加外源性人铁蛋白,并未显著增加结合抗铁蛋白抗体的单核细胞百分比。这些结果表明,铁过载的血色素沉着症患者的单核细胞表面铁蛋白表达增加,这可能代表铁蛋白的释放以及血色素沉着症特有的代谢缺陷。