Abstract Number: PB1306
Meeting: ISTH 2020 Congress
Background: Thalassemia disease is associated with thrombosis due to hypercoagulable state caused by several hemostatic derangements. Rotatory thromboelastometry (ROTEM®), a global hemostasis test, has been demonstrated hypercoagulable state in β-thalassemia/hemoglobin E disease (β/E). However, there are a few evidences in hemoglobin H disease (Hb H).
Aims: To compare the changes in ROTEM®, procoagulants and anticoagulants among children and adolescents with Hb H, β/E and controls.
Methods: We enrolled 38 subjects with Hb H, 22 subjects β/E with 12 age-matched controls. ROTEM®, D-dimer, procoagulants [factor VIII clotting activity (FVIII:C) and von Willebrand factor antigen (vWF:Ag)] and anticoagulants [antithrombin (AT), protein C (PC) and free protein S (FPS) activities] were performed in all subjects.
Results: The median age of all subjects was 13 years (min-max 5-21) and 54% were male. PC and FPS levels were significantly lower in Hb H compared with β/E and controls (83.5 VS 70.2 VS 112, P< 0.01; 57.6 VS 45.2 VS 86, P< 0.01, respectively). ROTEM® parameters were not statistically different among the all groups but maximum clot firmness (MCF) in EXTEM and INTEM was still significantly higher in splenectomized subjects than non-splenectomized subjects and controls (P< 0.01). PC and FPS levels in splenectomized patients were significantly higher than non-splenectomized subjects (P< 0.01) and were correlated with total and direct bilirubin and serum ferritin (r=-0.44, -0.35 and -0.41 for PC and r=-0.59, -0.38 and -0.54 for FPS, respectively; P< 0.01).
Conclusions: ROTEM® parameters were not different among Hb H, β/E and control groups in this study. Hypercoagulable state was more commonly found splenectomized subjects. Low PC and FPS likely caused by liver injury was frequently found in β/E than Hb H and control groups but were not associated with hypercoagulable state.
To cite this abstract in AMA style:Natesirinilkul R, Choedamphai C, Charoenkwan P, Sathitsamitphong L, Manowong S, Fanhchaksai K, Tachawong N. Global Hemostasis, Procoagulation and Anticoagulation Proteins Derangements in Children and Adolescents with Hemoglobin H and Beta-Thalassemia/Hemoglobin E Diseases [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/global-hemostasis-procoagulation-and-anticoagulation-proteins-derangements-in-children-and-adolescents-with-hemoglobin-h-and-beta-thalassemia-hemoglobin-e-diseases/. Accessed December 3, 2021.
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ISTH Congress Abstracts - https://abstracts.isth.org/abstract/global-hemostasis-procoagulation-and-anticoagulation-proteins-derangements-in-children-and-adolescents-with-hemoglobin-h-and-beta-thalassemia-hemoglobin-e-diseases/