Iron kinetics and erythropoiesis in Fanconi's anaemia.
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.
D000293:Adolescent; D000741:Anemia, Aplastic / Q000097:blood; D001853:Bone Marrow / Q000378:metabolism; D002648:Child; D002675:Child, Preschool; D004905:Erythrocyte Aging; D004912:Erythrocytes / Q000378:metabolism; D004920:Erythropoiesis; D005199:Fanconi Anemia / Q000097:blood / Q000378:metabolism / Q000503:physiopathology; D006454:Hemoglobins / Q000096:biosynthesis; D006801:Humans; D007501:Iron / Q000097:blood / Q000378:metabolism*
D006454:Hemoglobins; D007501:Iron