Samson D, Halliday D, Nicholson D C, Chanarin I
Br J Haematol. 1976 Sep;34(1):45-53. doi: 10.1111/j.1365-2141.1976.tb00172.x.
The incorporation of [15N]delta-aminolaevulinic acid and [15N glycine into haemoglobin haem and early labelled bilirubin was measured in subjects with various haematological disorders. The clearance of [14C bilirubin was used to measure bilirubin production rate, and the magnitude of the various sources of bilirubin production and the percentage ineffective erythropoiesis were calculated. Ineffective erythropoiesis was found to be a major factor in the production of the anaemia in patients with the following disorders: megaloblastic anaemia associated with the Lesch-Nyhan syndrome, thalassaemia intermedia, sideroblastic anaemia, and the anaemia of chronic disorders. In three patients with iron-deficiency anaemia ineffective erythropoiesis was increased, but was of minor importance in the production of the anaemia, while in two patients with aplastic anaemia and one with macrocytosis of alcoholism there was no increase in ineffective erythropoiesis.
在患有各种血液系统疾病的受试者中,测量了[15N]δ-氨基乙酰丙酸和[15N]甘氨酸掺入血红蛋白血红素和早期标记胆红素的情况。用[14C]胆红素的清除率来测量胆红素生成率,并计算胆红素生成的各种来源的大小以及无效红细胞生成的百分比。发现无效红细胞生成是以下疾病患者贫血产生的主要因素:与莱施-奈恩综合征相关的巨幼细胞贫血、中间型地中海贫血、铁粒幼细胞贫血和慢性疾病性贫血。在3例缺铁性贫血患者中,无效红细胞生成增加,但在贫血产生中占次要地位,而在2例再生障碍性贫血患者和1例酒精中毒性大细胞增多症患者中,无效红细胞生成没有增加。