Cazzola M, Alessandrino P, Barosi G, Morandi S, Stefanelli M
Scand J Haematol. 1979 Aug;23(2):107-14. doi: 10.1111/j.1600-0609.1979.tb02680.x.
Ferrokinetic studies were carried out in 8 patients with heterozygous beta-thalassaemia with anaemia of varying severity. Effective and ineffective erythropoiesis, mean red cell lifespan and non-erythroid iron turnover were estimated from the experimental data through a mathematical model of iron kinetics. Erythropoietic activity was markedly increased in all patients, but was variably ineffective (from 10 to 74%). A negative correlation (r = 0.855, P less than 0.01) was found between the amount of ineffective erythropoiesis and Hb level. Red cell lifespan was variably shortened and there was a negative correlation between the degree of daily peripheral haemolysis and Hb level (r = 0.733, P less than 0.05). Non-erythroid iron turnover was increased in most patients. The results provide quantitative measurements of the mechanisms responsible for the wide variation of the Hb level in heterozygous beta-thalaeeaemia. Ineffective erythropoiesis seems to be the major reason for the anaemia. Peripheral haemolysis contributes to it, especially in the most severely affected patients. The increased non-erythroid iron turnover may be responsible for the pathology which characterizes heterozygotes in the adult life.
对8例不同严重程度贫血的杂合子β地中海贫血患者进行了铁动力学研究。通过铁动力学数学模型，从实验数据中估算有效和无效红细胞生成、平均红细胞寿命及非红细胞铁周转率。所有患者的红细胞生成活性均显著增加，但有效性各异（从10%至74%）。无效红细胞生成量与血红蛋白水平之间呈负相关（r = 0.855，P < 0.01）。红细胞寿命有不同程度缩短，每日外周溶血程度与血红蛋白水平之间呈负相关（r = 0.733，P < 0.05）。大多数患者的非红细胞铁周转率增加。这些结果提供了对杂合子β地中海贫血患者血红蛋白水平广泛变化机制的定量测量。无效红细胞生成似乎是贫血的主要原因。外周溶血也有影响，尤其是在受影响最严重的患者中。非红细胞铁周转率增加可能是成年杂合子特征性病理改变的原因。
