Iron Kinetics and Erythropoiesis in Fanconi's Anaemia

Abstract

Ferrokinetic studies were carried out in 4 patients with Fanconi''s anemia (FA). Experimental data were analyzed by means of a mathematical model of iron kinetics 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 anemia 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. The pathogenesis and the natural course of the disease are discussed.