Chapman R W, Hussain M A, Gorman A, Laulicht M, Politis D, Flynn D M, Sherlock S, Hoffbrand A V
J Clin Pathol. 1982 May;35(5):487-91. doi: 10.1136/jcp.35.5.487.
The incidence of ascorbic acid (AA) deficiency and its effect on serum ferritin concentration relative to body iron stores was studied in 61 unchelated patients with beta-thalassaemia major. Thirty-nine (64%) of patients had subnormal leucocyte ascorbate concentrations without clinical evidence of scurvy. The lowest leucocyte ascorbate concentrations tended to occur in the most transfused patients. No correlation was found between the units transfused and serum ferritin concentration in the AA-deficient patients but a close correlation (r = +0.82; p less than 0.005) existed for the AA-replete group. Similarly a close correlation (r = +0.77; p less than 0.005) was obtained between liver iron concentration and serum ferritin in AA-replete patients but only a weak correlation (r = +0.385; p less than 0.025) existed for the AA-deficient group. When AA-deficient patients were treated with ascorbic acid, serum iron and percentage saturation of iron binding capacity rose significantly; serum ferritin rose in 13 of 21 patients despite the simultaneous commencement of desferrioxamine therapy. In contrast all three measurements tended to fall in AA-replete patients with ascorbic acid and desferrioxamine therapy. Thus, AA deficiency is commonly present in beta-thalassaemia patients with iron overload and may give rise to inappropriate serum ferritin concentrations in relation to body iron stores.
对61例未经螯合治疗的重型β地中海贫血患者,研究了抗坏血酸(AA)缺乏的发生率及其对血清铁蛋白浓度与机体铁储备关系的影响。39例(64%)患者白细胞抗坏血酸浓度低于正常,无坏血病的临床证据。白细胞抗坏血酸浓度最低者往往是输血最多的患者。在AA缺乏的患者中,输血单位数与血清铁蛋白浓度之间未发现相关性,但在AA充足的组中存在密切相关性(r = +0.82;p<0.005)。同样,在AA充足的患者中,肝脏铁浓度与血清铁蛋白之间也存在密切相关性(r = +0.77;p<0.005),但在AA缺乏的组中仅存在弱相关性(r = +0.385;p<0.025)。当对AA缺乏的患者用抗坏血酸治疗时,血清铁和铁结合能力的百分比饱和度显著升高;尽管同时开始去铁胺治疗,但21例患者中有13例血清铁蛋白升高。相比之下,在接受抗坏血酸和去铁胺治疗的AA充足的患者中,所有这三项测量值往往都会下降。因此,AA缺乏在铁过载的β地中海贫血患者中普遍存在,并且可能导致与机体铁储备相关的血清铁蛋白浓度异常。