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