Background:Â Thalassemia is the most common hereditary blood disorder in the world and Indonesia.Â MajorÂ Î²-thalassemiaÂ is classified asÂ Transfusion-Dependent Thalassemia (TDT).Â Specific changes in the composition of red cell membrane lipids and hemosiderosis may lead to hypercoagulation. This study aimed to determine the difference in Protein C, Protein S, Antithrombin III (AT-III), and D-dimer between majorÂ Î²-thalassemiaÂ patients and normal people.
Methods:Â This was an observational analytical study with a cross-sectional design, including 18 major Î²-thalassemia patients in H. Adam Malik Hospital Medan who met the study criteria and 18 normal controls. Subjects were examined for Protein C, Protein S, AT-III and D-dimer. The assessment was also carried out among patients based on their transfusion frequency and serum ferritin.
Results: There were significant differences in Protein C, Protein S, and D-dimer levels between majorÂ Î²-thalassemiaÂ patients and normal controls (p < 0.05). AT-III level in majorÂ Î²-thalassemiaÂ patients was lower than healthy controls, however the difference was not significant. The level of Protein C in patients who infrequently and frequently underwent blood transfusions were 34.88 Â± 8.6 and 45.19 Â± 7.8 respectively, which was significantly different (p < 0.05). A significant differences in Protein C, Protein S and D-dimer were also found between patients with serum ferritin level < 2,500 ng/ml and Â â‰¥ 2500 ng/ml.
Conclusion:Â Significant changes were found in anticoagulation proteins of major Î²-thalassemia patients. Frequent blood transfusions and chelation therapy should be emphasized for major thalassemia patients because they affect anticoagulation protein levels.