Congenital Dyserythropoietic Anemia Type I Clinical Trial
Official title:
The Impact of Growth Differentiating Factor (GDF) 15 in Sickle Cell Disease and Hereditary Spherocytosis
Patients with thalassemia intermedia, congenital dyserythropoietic anemia type I , and sideroblastic anemia were found to express very high levels of serum GDF15, and this contributed to the inappropriate suppression of hepcidin with subsequent secondary iron overload.The aim of our present study is to asses the levels of GDF15 and hepcidin in patients with Sickle cell disease and hereditary spherocytosis
The identification of the ferroportin/hepcidin axis has allowed the effect of erythroid
activity on iron balance to be studied and has created the basis for better defining the
erythroid regulators.
In iron-loading anemias, ineffective erythropoiesis suppresses hepcidin production, which
result in dysregulating iron homeostasis. Miller and co-workers showed that release of
cytokines like growth differentiation factor 15 (GDF15) during the process of ineffective
erythropoiesis inhibits hepcidin production, thus defining a molecular link between
ineffective erythropoiesis, suppression of hepcidin production and parenchymal iron loading.
;
Time Perspective: Prospective