title: Thromboelastometry profile in children with beta-thalassemia.
abstract: 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.
authors.affiliation: Division of Pediatric Hematology and Oncology, Department of Pediatrics, Eskisehir Osmangazi University Faculty of Medicine, Eskisehir, 26480, Turkey, aysebturhan@hotmail.com.
authors.forename: Ayşe B
authors.initials: AB
authors.lastname: Turhan
authors.forename: Özcan
authors.initials: Ö
authors.lastname: Bör
authors.forename: O Meltem
authors.initials: OM
authors.lastname: Akay
authors.forename: Necat A
authors.initials: NA
authors.lastname: Akgün
mesh_terms: D000293:Adolescent; D001777:Blood Coagulation; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D013156:Splenectomy; D013916:Thrombelastography / Q000379:methods*; D017086:beta-Thalassemia / Q000097:blood* / Q000175:diagnosis* / Q000601:surgery