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

Area of Science:

• Hematology
• Thrombosis Research
• Genetic Blood Disorders

Background:

• Beta-thalassemia major (β-TM) is an inherited blood disorder impacting beta-globin production.
• A complication of β-TM is thrombosis, particularly in transfusion-dependent patients.
• Reduced levels of natural anticoagulants like protein C (PC), protein S (PS), and antithrombin (AT) may contribute to thrombosis risk.

Purpose of the Study:

• To investigate the levels of natural anticoagulants in patients with β-TM.
• To assess the potential link between transfusion history and anticoagulant levels in β-TM.
• To evaluate the association between decreased anticoagulant levels and thrombosis risk in β-TM.

Main Methods:

• A case-control study was conducted.
• 118 patients with β-TM (receiving ≥20 transfusions) and 120 healthy controls were included.
• Exclusion criteria included other bleeding or thrombotic disorders.

Main Results:

• Mean PC and AT levels were significantly lower in β-TM patients compared to controls (P=0.001 and P=0.01, respectively).
• Total PS levels showed a similar trend, though not statistically significant (P=0.1).
• PC, AT, and total PS levels were decreased by 50.36%, 27.5%, and 15.34%, respectively, in β-TM patients.

Conclusions:

• Prolonged, frequent blood transfusions in β-TM patients are associated with decreased natural anticoagulant levels.
• These reductions in PC, PS, and AT may increase the risk of thrombosis in β-TM patients.
• Further research is warranted to explore prophylactic strategies against thrombosis in this population.