Skip to main content
Top
Published in: International Journal of Hematology 4/2014

01-04-2014 | Original Article

Thromboelastometry profile in children with beta-thalassemia

Authors: Ayşe B. Turhan, Özcan Bör, O. Meltem Akay, Necat A. Akgün

Published in: International Journal of Hematology | Issue 4/2014

Login to get access

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.
Literature
1.
go back to reference Taher AT, Musallam KM, Cappellini MD, Weatherall DJ. Optimal management of β thalassaemia intermedia. Br J Haematol. 2011;152:512–23.PubMedCrossRef Taher AT, Musallam KM, Cappellini MD, Weatherall DJ. Optimal management of β thalassaemia intermedia. Br J Haematol. 2011;152:512–23.PubMedCrossRef
2.
3.
go back to reference Winichagoon P, Fucharoen S, Wasi P. Increased circulating platelet aggregates in thalassaemia. Southeast Asian J Trop Med Public Health. 1981;12:556–60.PubMed Winichagoon P, Fucharoen S, Wasi P. Increased circulating platelet aggregates in thalassaemia. Southeast Asian J Trop Med Public Health. 1981;12:556–60.PubMed
4.
go back to reference Shinar E, Rachmilewitz EA, Lux SE. Differing erythrocyte membrane skeletal protein defects in alpha and beta thalassemia. J Clin Invest. 1989;83:404–10.PubMedCentralPubMedCrossRef Shinar E, Rachmilewitz EA, Lux SE. Differing erythrocyte membrane skeletal protein defects in alpha and beta thalassemia. J Clin Invest. 1989;83:404–10.PubMedCentralPubMedCrossRef
5.
go back to reference Musallam KM, Taher AT. Thrombosis in thalassemia: why are we so concerned? Hemoglobin. 2011;35:503–10.PubMedCrossRef Musallam KM, Taher AT. Thrombosis in thalassemia: why are we so concerned? Hemoglobin. 2011;35:503–10.PubMedCrossRef
6.
go back to reference Spiezia L, Marchioro P, Radu C, Rossetto V, Tognin G, Monica C, Salmaso L, Simioni P. Whole blood coagulation assessment using rotation thrombelastogram thromboelastometry in patients with acute deep vein thrombosis. Blood Coagul Fibrinolysis. 2008;19:355–60.PubMedCrossRef Spiezia L, Marchioro P, Radu C, Rossetto V, Tognin G, Monica C, Salmaso L, Simioni P. Whole blood coagulation assessment using rotation thrombelastogram thromboelastometry in patients with acute deep vein thrombosis. Blood Coagul Fibrinolysis. 2008;19:355–60.PubMedCrossRef
7.
go back to reference Hvitfeldt Poulsen L, Christiansen K, Sorensen B, Ingerslev J. Whole blood thrombelastographic coagulation profiles using minimal tissue factor activation can display hypercoagulation in thrombosis-prone patients. Scand J Clin Lab Invest. 2006;66:329–36.PubMedCrossRef Hvitfeldt Poulsen L, Christiansen K, Sorensen B, Ingerslev J. Whole blood thrombelastographic coagulation profiles using minimal tissue factor activation can display hypercoagulation in thrombosis-prone patients. Scand J Clin Lab Invest. 2006;66:329–36.PubMedCrossRef
8.
go back to reference Sorensen B, Johansen P, Christiansen K, Woelke M, Ingerslev J. Whole blood coagulation thrombelastographic profiles employing minimal tissue factor activation. J Thromb Haemost. 2003;1:551–8.PubMedCrossRef Sorensen B, Johansen P, Christiansen K, Woelke M, Ingerslev J. Whole blood coagulation thrombelastographic profiles employing minimal tissue factor activation. J Thromb Haemost. 2003;1:551–8.PubMedCrossRef
9.
go back to reference Rossetto V, Spiezia L, Senzolo M, Rodriguez-Castro KI, Maggiolo S, Simioni P. Whole blood rotation thromboelastometry (ROTEM®) profiles in subjects with non-neoplastic portal vein thrombosis. Thromb Res. 2013;132:e131–4.PubMedCrossRef Rossetto V, Spiezia L, Senzolo M, Rodriguez-Castro KI, Maggiolo S, Simioni P. Whole blood rotation thromboelastometry (ROTEM®) profiles in subjects with non-neoplastic portal vein thrombosis. Thromb Res. 2013;132:e131–4.PubMedCrossRef
10.
go back to reference Ko RH, Ji L, Young G. A novel approach for detecting hypercoagulability utilizing thromboelastography. Thromb Res. 2013;131:352–6.PubMedCrossRef Ko RH, Ji L, Young G. A novel approach for detecting hypercoagulability utilizing thromboelastography. Thromb Res. 2013;131:352–6.PubMedCrossRef
11.
go back to reference Yee DL, Edwards RM, Mueller BU, Teruya J. Thromboelastographic and hemostatic characteristics in pediatric patients with sickle cell disease. Arch Pathol Lab Med. 2005;129:760–5.PubMed Yee DL, Edwards RM, Mueller BU, Teruya J. Thromboelastographic and hemostatic characteristics in pediatric patients with sickle cell disease. Arch Pathol Lab Med. 2005;129:760–5.PubMed
12.
go back to reference Papa ML, Capasso F, Pudore L, Torre S, Mango S, Russo V, Delrio P, Palaia R, Ruffolo F, d’Eufemia MD, De Lucia D, Napolitano M, Di Micco P, Parisi V. Thromboelastographic profiles as a tool for thrombotic risk in digestive tract cancer. Exp Oncol. 2007;29:111–5.PubMed Papa ML, Capasso F, Pudore L, Torre S, Mango S, Russo V, Delrio P, Palaia R, Ruffolo F, d’Eufemia MD, De Lucia D, Napolitano M, Di Micco P, Parisi V. Thromboelastographic profiles as a tool for thrombotic risk in digestive tract cancer. Exp Oncol. 2007;29:111–5.PubMed
13.
go back to reference Nielsen VG, Geary BT, Baird MS. Evaluation of the contribution of platelets to clot strength by thromboelastography in rabbits: the role of tissue factor and cytochalasin D. Anesth Analg. 2000;91:35–9.PubMed Nielsen VG, Geary BT, Baird MS. Evaluation of the contribution of platelets to clot strength by thromboelastography in rabbits: the role of tissue factor and cytochalasin D. Anesth Analg. 2000;91:35–9.PubMed
14.
go back to reference Zuckerman L, Cohen E, Vagher JP, Woodward E, Caprini JA. Comparison of thrombelastography with common coagulation tests. Thromb Haemost. 1981;46:752–6.PubMed Zuckerman L, Cohen E, Vagher JP, Woodward E, Caprini JA. Comparison of thrombelastography with common coagulation tests. Thromb Haemost. 1981;46:752–6.PubMed
15.
go back to reference Zurlo MG, De Stefano P, Borgna-Pignatti C, Di Palma A, Piga A, Melevendi C, Di Gregorio F, Burattini MG, Terzoli S. Survival and causes of death in thalassaemia major. Lancet. 1989;2:27–30.PubMedCrossRef Zurlo MG, De Stefano P, Borgna-Pignatti C, Di Palma A, Piga A, Melevendi C, Di Gregorio F, Burattini MG, Terzoli S. Survival and causes of death in thalassaemia major. Lancet. 1989;2:27–30.PubMedCrossRef
16.
go back to reference Borgna Pignatti C, Carnelli V, Caruso V, Dore F, De Mattia D, Di Palma A, Di Gregorio F, Romeo MA, Longhi R, Mangiagli A, Melevendi C, Pizzarelli G, Musumeci S. Thromboembolic events in beta thalassemia major: an Italian multicenter study. Acta Haematol. 1998;99:76–9.PubMedCrossRef Borgna Pignatti C, Carnelli V, Caruso V, Dore F, De Mattia D, Di Palma A, Di Gregorio F, Romeo MA, Longhi R, Mangiagli A, Melevendi C, Pizzarelli G, Musumeci S. Thromboembolic events in beta thalassemia major: an Italian multicenter study. Acta Haematol. 1998;99:76–9.PubMedCrossRef
17.
go back to reference Cappellini MD, Robbiolo L, Bottasso BM, Coppola R, Fiorelli G, Mannucci AP. Venous thromboembolism and hypercoagulability in splenectomized patients with thalassaemia intermedia. Br J Haematol. 2000;111:467–73.PubMedCrossRef Cappellini MD, Robbiolo L, Bottasso BM, Coppola R, Fiorelli G, Mannucci AP. Venous thromboembolism and hypercoagulability in splenectomized patients with thalassaemia intermedia. Br J Haematol. 2000;111:467–73.PubMedCrossRef
18.
go back to reference Cappellini MD, Poggiali E, Taher AT, Musallam KM. Hypercoagulability in β-thalassemia: a status quo. Expert Rev Hematol. 2012;5:505–11.PubMedCrossRef Cappellini MD, Poggiali E, Taher AT, Musallam KM. Hypercoagulability in β-thalassemia: a status quo. Expert Rev Hematol. 2012;5:505–11.PubMedCrossRef
19.
go back to reference Goldschmidt N, Spectre G, Brill A, Zelig O, Goldfarb A, Rachmilewitz E, Varon D. Increased platelet adhesion under flow conditions is induced by both thalassemic platelets and red blood cells. Thromb Haemost. 2008;100:864–70.PubMed Goldschmidt N, Spectre G, Brill A, Zelig O, Goldfarb A, Rachmilewitz E, Varon D. Increased platelet adhesion under flow conditions is induced by both thalassemic platelets and red blood cells. Thromb Haemost. 2008;100:864–70.PubMed
20.
go back to reference Butthep P, Bunyaratvej A, Funahara Y, Kitaguchi H, Fucharoen S, Sato S, Bhamarapravati N. Alterations in vascular endothelial cell-related plasma proteins in thalassaemic patients and their correlation with clinical symptoms. Thromb Haemost. 1995;74:1045–9.PubMed Butthep P, Bunyaratvej A, Funahara Y, Kitaguchi H, Fucharoen S, Sato S, Bhamarapravati N. Alterations in vascular endothelial cell-related plasma proteins in thalassaemic patients and their correlation with clinical symptoms. Thromb Haemost. 1995;74:1045–9.PubMed
21.
go back to reference Taher AT, Otrock ZK, Uthman I, Cappellini MD. Thalassemia and hypercoagulability. Blood Rev. 2008;22:283–92.PubMedCrossRef Taher AT, Otrock ZK, Uthman I, Cappellini MD. Thalassemia and hypercoagulability. Blood Rev. 2008;22:283–92.PubMedCrossRef
22.
go back to reference Cappellini MD, Motta I, Musallam KM, Taher AT. Redefining thalassemia as a hypercoagulable state. Ann N Y Acad Sci. 2010;1202:231–6.PubMedCrossRef Cappellini MD, Motta I, Musallam KM, Taher AT. Redefining thalassemia as a hypercoagulable state. Ann N Y Acad Sci. 2010;1202:231–6.PubMedCrossRef
23.
go back to reference Ataga KI, Cappellini MD, Rachmilewitz EA. Beta-thalassaemia and sickle cell anaemia as paradigms of hypercoagulability. Br J Haematol. 2007;139:3–13.PubMedCrossRef Ataga KI, Cappellini MD, Rachmilewitz EA. Beta-thalassaemia and sickle cell anaemia as paradigms of hypercoagulability. Br J Haematol. 2007;139:3–13.PubMedCrossRef
24.
go back to reference Tripodi A, Cappellini MD, Chantarangkul V, Padovan L, Fasulo MR, Marcon A, Mannucci PM. Hypercoagulability in splenectomized thalassemic patients detected by whole-blood thromboelastometry, but not by thrombin generation in platelet-poor plasma. Haematologica. 2009;94:1520–7.PubMedCentralPubMedCrossRef Tripodi A, Cappellini MD, Chantarangkul V, Padovan L, Fasulo MR, Marcon A, Mannucci PM. Hypercoagulability in splenectomized thalassemic patients detected by whole-blood thromboelastometry, but not by thrombin generation in platelet-poor plasma. Haematologica. 2009;94:1520–7.PubMedCentralPubMedCrossRef
25.
go back to reference Afshari A, Wikkelsø A, Brok J, Møller AM, Wetterslev J. Thrombelastography (TEG) or thromboelastometry (ROTEM) to monitor haemotherapy versus usual care in patients with massive transfusion. Cochrane Database Syst Rev. 2011;2011:007871. Afshari A, Wikkelsø A, Brok J, Møller AM, Wetterslev J. Thrombelastography (TEG) or thromboelastometry (ROTEM) to monitor haemotherapy versus usual care in patients with massive transfusion. Cochrane Database Syst Rev. 2011;2011:007871.
26.
go back to reference Di Benedetto P, Baciarello M, Cabetti L, Martucci M, Chiaschi A, Bertini L. Thrombelastography. Present and future perspectives in clinical practice. Minerva Anestesiol. 2003;69:501–15.PubMed Di Benedetto P, Baciarello M, Cabetti L, Martucci M, Chiaschi A, Bertini L. Thrombelastography. Present and future perspectives in clinical practice. Minerva Anestesiol. 2003;69:501–15.PubMed
27.
29.
go back to reference Keawvichit R, Khowawisetsut L, Chaichompoo P, Polsrila K, Sukklad S, Sukapirom K, Khuhapinant A, Fucharoen S, Pattanapanyasat K. Platelet activation and platelet–leukocyte interaction in β-thalassemia/hemoglobin E patients with marked nucleated erythrocytosis. Ann Hematol. 2012;91:1685–94.PubMedCrossRef Keawvichit R, Khowawisetsut L, Chaichompoo P, Polsrila K, Sukklad S, Sukapirom K, Khuhapinant A, Fucharoen S, Pattanapanyasat K. Platelet activation and platelet–leukocyte interaction in β-thalassemia/hemoglobin E patients with marked nucleated erythrocytosis. Ann Hematol. 2012;91:1685–94.PubMedCrossRef
Metadata
Title
Thromboelastometry profile in children with beta-thalassemia
Authors
Ayşe B. Turhan
Özcan Bör
O. Meltem Akay
Necat A. Akgün
Publication date
01-04-2014
Publisher
Springer Japan
Published in
International Journal of Hematology / Issue 4/2014
Print ISSN: 0925-5710
Electronic ISSN: 1865-3774
DOI
https://doi.org/10.1007/s12185-014-1546-6

Other articles of this Issue 4/2014

International Journal of Hematology 4/2014 Go to the issue
Webinar | 19-02-2024 | 17:30 (CET)

Keynote webinar | Spotlight on antibody–drug conjugates in cancer

Antibody–drug conjugates (ADCs) are novel agents that have shown promise across multiple tumor types. Explore the current landscape of ADCs in breast and lung cancer with our experts, and gain insights into the mechanism of action, key clinical trials data, existing challenges, and future directions.

Dr. Véronique Diéras
Prof. Fabrice Barlesi
Developed by: Springer Medicine