范科尼贫血中的铁动力学与红细胞生成

Iron kinetics and erythropoiesis in Fanconi's anaemia.

作者信息

Barosi G, Cazzola M, Marchi A, Morandi S, Perani V, Stefanelli M, Perugini S

出版信息

Scand J Haematol. 1978 Jul;21(1):29-39. doi: 10.1111/j.1600-0609.1978.tb02492.x.

DOI:10.1111/j.1600-0609.1978.tb02492.x

Abstract

Ferrokinetic studies were carried out in 4 patients with Fanconi's anaemia (FA). Experimental data were analysed by means of a mathematical model of iron kinetics in order to obtain a quantitative assessment of effective and ineffective erythropoietic activity, mean red cell lifespan, and non-erythroid iron turnover. The major pathogenetic mechanism of the anaemia appeared to be relative marrow failure, i.e. a reduction in the proliferative capacity of the erythroid marrow. The role of ineffective erythropoiesis was of minor importance. On the basis of the results obtained both the pathogenesis and the natural course of the disease are discussed.

摘要

对4例范可尼贫血（FA）患者进行了铁动力学研究。通过铁动力学数学模型对实验数据进行分析，以定量评估有效和无效的红细胞生成活性、平均红细胞寿命以及非红细胞铁周转率。贫血的主要发病机制似乎是相对骨髓衰竭，即红系骨髓增殖能力降低。无效红细胞生成的作用较小。根据所得结果，对该疾病的发病机制和自然病程进行了讨论。