Soluble hemojuvelin in transfused and untransfused thalassaemic subjects

Objective The hemojuvelin-bone morphogenetic protein axis is the principal iron-dependent mechanism of hepcidin regulation. The determination of sHJV levels could allow for a better understanding of the pathophysiological mechanisms of hepcidin regulation in thalassaemia. Method We have assessed soluble hemojuvelin (sHJV) in 45 transfused and 15 untransfused thalassaemic patients in comparison to 15 healthy subjects, evaluating its relationships with some parameters of iron overload, anaemia and erythropoiesis. Results Untransfused thalassaemic patients had more severe anaemia and erythropoietic activity, while in transfused patients, the transfused RBCs reduced % reticulocytes and sTfR, increased serum indices of iron overload and iron stores in the liver (low RMI T2* values). sHJV levels were higher in patients than in controls and in untransfused in comparison to transfused patients. In the transfused group, we also found that sHJV values are significantly related to serum ferritin, cardiac MRI T2* and growth differentiation factor 15 and are sensitive to hepatitis C virus infection. Conclusion These results suggest that sHJV synthesis seems to be affected by an erythropoietic/hypoxic signal in untransfused patients that have severe anaemia, while in regularly transfused subjects, it is influenced by iron stores. This article is protected by copyright. All rights reserved.