Thromboelastometry profile in children with beta-thalassemia

Beta (β)-thalassemia is characterized by a hypercoagulable state and an increased risk of thrombosis, which can result in significant morbidity and mortality. The coagulation pattern and determinants of thrombosis in patients with β-thalassemia remain largely unknown. The aim of this study was to evaluate the whole blood thromboelastometry (TEM) profile of β-thalassemic children by ROTEM^®. ROTEM^® assays (INTEM, EXTEM) and traditional coagulation parameters (platelet count, prothrombin time, activated partial thromboplastin time, and fibrinogen) were performed on blood samples from 17 subjects with β-thalassemia and 19 non-thalassemic controls. Maximum clot firmness (MCF) was significantly higher in subjects with β-thalassemia than in controls on EXTEM and INTEM analysis (p < 0.001 and p < 0.001, respectively). Of the patients with β-thalassemia, MCF was higher and clot formation time was shorter in splenectomized subjects than in non-splenectomized subjects on EXTEM and INTEM (p = 0.026, p = 0.002, p < 0.001, p < 0.001, respectively). TEM profiles in β-thalassemic children were more hypercoagulable compared with controls. Larger prospective studies are needed to evaluate the relevance of the association between ROTEM^® profile and thromboembolic events in patients with β-thalassemia.