HYPERCOAGULABILITY AND THROMBOTIC RISK IN BETA-THALASSEMIA: CLINICAL AND LABORATORY INSIGHTS

Snezhana Stoencheva

doi:10.35120/medisij0501033s

Authors

Snezhana Stoencheva Department of Clinical Laboratory, Faculty of Medicine, Medical University of Plovdiv, University Hospital “St. George”, Plovdiv, Bulgaria Author

DOI:

https://doi.org/10.35120/medisij0501033s

Keywords:

Beta-thalassemia, hypercoagulability, thrombotic risk, iron overload

Abstract

Beta-thalassemia is increasingly recognized as a multisystem disorder associated with a chronic hypercoagulable state and an elevated risk of thrombotic complications. Beyond ineffective erythropoiesis, chronic anemia, and transfusion-related iron overload, abnormalities in coagulation and vascular function play an important role in long-term morbidity. Accumulating evidence indicates that patients with beta-thalassemia exhibit persistent activation of cellular and plasma components of hemostasis, even in the absence of overt clinical thrombosis. Hypercoagulability in beta-thalassemia is multifactorial, arising from altered red blood cell membrane architecture with phosphatidylserine externalization, chronic platelet activation, endothelial dysfunction driven by hemolysis and nitric oxide depletion, increased circulating procoagulant microparticles and extracellular vesicles, and imbalances in natural anticoagulant pathways. Iron-related oxidative stress and inflammation further amplify vascular activation and thrombin generation. The magnitude and clinical expression of these abnormalities differ substantially across disease phenotypes. Beta-thalassemia trait is generally not associated with clinically significant thrombosis, whereas non-transfusion-dependent thalassemia (NTDT) carries a particularly high thrombotic burden, especially in splenectomized patients. In transfusion-dependent thalassemia (TDT), regular transfusion modifies erythroid-driven procoagulant mechanisms but introduces additional risk modifiers, including iron overload and catheter-related thrombosis. This review summarizes current knowledge on coagulation disturbances and thrombotic complications in beta-thalassemia, integrating pathophysiological mechanisms with clinically relevant laboratory markers. Particular emphasis is placed on global coagulation assays, cellular biomarkers, and phenotype-specific clinical-laboratory patterns, as well as on contemporary monitoring strategies in TDT. A better understanding of these mechanisms is essential for individualized risk stratification and optimization of thrombosis prevention in patients with beta-thalassemia.

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