Natural Anticoagulant Protein Levels in Patients With Beta-Thalassemia Major: A Case-Control Study

Abstract

Background: ��-thalassemia is a group of inherited blood disorders that affect the production of ��-globin chains, leading to the reduction or absence of these chains. One of the complications observed in patients with ��-thalassemia major (��-TM) is thrombosis, especially in those who receive frequent blood transfusions. This may be due to a decrease in the levels of the natural anticoagulants: protein C (PC), total protein S (PS), and antithrombin (AT). Methods: In this case-control study, patients with ��-TM, who had received at least 20 packed cell transfusions during their lifetime, were included. Patients with other underlying diseases like bleeding or thrombotic disorders were excluded. Totally, 118 patients with ��-TM and 120 healthy individuals were included. Results: The mean level of PC and AT was significantly lower in patients with ��-TM (48.2 �� 65.4 and 57.42 �� 13.6, respectively) compared to the control group (97.1 �� 21.46 and 81.79 �� 14.3, respectively), with P value of 0.001 and 0.01, respectively. Although the difference was not statistically significant (P = 0.1), a similar trend was observed for total PS (61.12 �� 21.12 for patients versus 72.2 �� 35.2 for the control group). Of note, the decrease in PC, AT, and total PS levels compared to the control group was 50.36%, 27.5%, and 15.34%, respectively. Conclusions: It seems that ��-TM patients who receive prolonged blood transfusions frequently are at an increased risk of decreased in natural anticoagulants levels and therefore potentially are at risk of thrombosis. J Hematol. 2024;13(1-2):23-28 doi: https://doi.org/10.14740/jh1217