Soluble transferrin receptor as a potential determinant of iron loading incongenital anaemias due to ineffective erythropoiesis

Congenital anaemias due to ineffective erythropoiesis may be associated wit h excessive iron absorption and progressive iron loading. We investigated w hether the soluble transferrin receptor (TfR) level was related to the degr ee of iron overload in 20 patients with thalassaemia intermedia, six patien ts with congenital dyserythropoietic anaemia type II (CDA II) and four pati ents with X-linked congenital sideroblastic anaemia (XLSA). All but two pat ients had increased serum ferritin levels (median 601 mu g/l, range 105-285 5 mu g/l). Multiple regression analysis showed that 62% (P<0.0001) of the v ariation in serum ferritin was explained by age and by changes in soluble T fR.