Assessment of ferritin and hepcidin levels in splenectomised and non-splenectomised β-thalassemia major patients and exploring a potential correlation with von Willebrand factor and ADAMTS-13

Abstract

Iron overload is a major complication in β-thalassemia major (β-TM) patients, resulting from ineffective erythropoiesis, increased gastrointestinal iron absorption and multiple blood transfusions. Excess iron accumulates in various organs, leading to organ dysfunction, and increased risk of thrombotic events. In this study we aim to determine levels of ferritin and its regulation hormone hepcidin in multi-transfused splenectomised and non-splenectomised β-thalassemia major patients and assess a possible correlation with the coagulation protein von Willebrand factor (vWF) and its cleaving protease ADAMTS-13. The study was conducted on 80 β-thalassemia major patients and 80 age- and sex-matched healthy controls. Plasma levels of vWF, ADAMTS-13, and hepcidin were assessed using the ELISA method. All patients presented with significantly higher levels of ferritin compared to normal controls (p < 0.001), while hepcidin levels were barely higher in patients (p = 0.05). Ferritin had a positive correlation with vWF antigen levels (r = 0.222, p = 0.05), ADAMTS-13 antigen levels (r = 0.334, p = 0.002) and ADAMTS-13 activity levels (r = 0.353, p = 0.001) in patients. Splenectomised patients had significantly higher levels of white blood cell counts, platelet counts and vWF antigen levels compared to non-splenectomised patients (p < 0.05), but ferritin and hepcidin levels were comparable between the two groups (p > 0.05). Hepcidin was not found to be correlated with any of the measured parameters in patients (p > 0.05). Iron overload is well manifested in our study group despite continuous chelation therapy. Unlike hepcidin, ferritin appeared to be associated with increased secretion of vWF and ADAMTS-13 in patients, while splenectomy had no effect on ferritin or hepcidin levels. These findings highlight the importance of proper iron monitoring in β-TM and recognition of thrombotic risks in managing this anemia.

Keywords: Hepcidin; Hypercoagulability; Iron overload; Splenectomy; Thalassemia.

Fig. 1

A scatter plot showing correlation between ferritin and vWF: Collagen-Binding Activity, vWF: Antigen, ADAMTIS-13 Activity and ADAMTIS-13: Antigen in β-Thalassemia major patients

Fig. 2

A scatter plot showing correlation between ferritin and vWF: Collagen-Binding Activity, vWF: Antigen, ADAMTIS-13 Activity and ADAMTIS-13: Antigen in in spelenectomized and non-splenectomized β-Thalassemia major patients