ORIGINAL ARTICLE

The hypercoagulation state among major β-thalassemia patients at H. Adam Malik Hospital, Medan, Indonesia

Jane Tetraulina Silitonga , Adi Koesoema Aman, Bidasari Lubis

Jane Tetraulina Silitonga
Department of Clinical Pathology, Faculty of Medicine, Universitas Sumatera Utara, H.Adam Malik Hospital, Medan, Indonesia. Email: tetra.jane@gmail.com

Adi Koesoema Aman
Department of Clinical Pathology, Faculty of Medicine, Universitas Sumatera Utara, H.Adam Malik Hospital, Medan, Indonesia

Bidasari Lubis
Department of Pediatrics, Faculty of Medicine, Universitas Sumatera Utara, H. Adam Malik Hospital, Medan, Indonesia
Online First: August 01, 2019 | Cite this Article
Silitonga, J., Aman, A., Lubis, B. 2019. The hypercoagulation state among major β-thalassemia patients at H. Adam Malik Hospital, Medan, Indonesia. Bali Medical Journal 8(2). DOI:10.15562/bmj.v8i2.1400


Background: Thalassemia is the most common hereditary blood disorder in the world and Indonesia. Major β-thalassemia is classified as Transfusion-Dependent Thalassemia (TDT). Specific changes in the composition of red cell membrane lipids and hemosiderosis may lead to hypercoagulation. This study aimed to determine the difference in Protein C, Protein S, Antithrombin III (AT-III), and D-dimer between major β-thalassemia patients and normal people.

Methods: This was an observational analytical study with a cross-sectional design, including 18 major β-thalassemia patients in H. Adam Malik Hospital Medan who met the study criteria and 18 normal controls. Subjects were examined for Protein C, Protein S, AT-III and D-dimer. The assessment was also carried out among patients based on their transfusion frequency and serum ferritin.

Results: There were significant differences in Protein C, Protein S, and D-dimer levels between major β-thalassemia patients and normal controls (p < 0.05). AT-III level in major β-thalassemia patients was lower than healthy controls, however the difference was not significant. The level of Protein C in patients who infrequently and frequently underwent blood transfusions were 34.88 ± 8.6 and 45.19 ± 7.8 respectively, which was significantly different (p < 0.05). A significant differences in Protein C, Protein S and D-dimer were also found between patients with serum ferritin level < 2,500 ng/ml and  ≥ 2500 ng/ml.

Conclusion: Significant changes were found in anticoagulation proteins of major β-thalassemia patients. Frequent blood transfusions and chelation therapy should be emphasized for major thalassemia patients because they affect anticoagulation protein levels.

References

Rachmilewitz EA, Giardina PJ. How I treat thalassemia. Blood. 2011; 118(13): 3479 – 88.

Kementerian Kesehatan Republik Indonesia. Kondisi Terkini Thalassemia di Indonesia. Jakarta: Kemenkes RI; 2007.

Viprakasit V, Origa R. Genetic Basis, Pathophysiology and Diagnosis. In: Cappellini MD, Cohen A, Porter J, Taher A, Viprakasit V. (eds.) Guidelines for the Management of Transfusion Dependent Thalassemia. 3rd ed. Cyprus: Thalassemia International Federation; 2014: 14-26.

Musumeci S, Leonardi S, Di Dio R, Fischer A, Di Costa G. Protein C and antithrombin III in polytransfused thalassemic patients. Acta Haematol. 1987; 77(1): 30–3.

Eldor A, Rachmilewitz EA. The hypercoagulable state in thalassemia. Blood. 2002; 99(1):36–43.

Mabood SAE, Fahmy DM, Akef A, Sallab SE. Protein C and Anti-Thrombin-III Deficiency in Children With Beta Thalassemia. J Hematol. 2018; 7(2):62–68.

Thalassemia International Federation. About Alpha Thalassemia. In: Hemoglobin Disorders Hemoglobinopathies. Cyprus: Thalassemia International Federation; 2007.

Kuypers FA, Lewis RA, Hua M, Schott MA, Discher D, Ernst JD, Lubin BH. Detection of altered membrane phospholipid asymmetry in subpopulations of human red blood cells using fluorescently labeled annexin V. Blood. 1996; 87(3):1179–87.

Eldor A, Krausz Y, Atlan H, Snyder D, Goldfarb A, Hy-Am E et al. Platelet survival in patients with beta-thalassemia. Am J Hematol. 1989; 32(2):94–9.

Hassan TH, Elbehedy RM, Youssef DM, Amr GE. Protein C levels in beta-thalassemia major patients in the east Nile delta of Egypt. Hematol Oncol Stem Cell Ther. 2010; 3(2):60–5.

Dahlback B. The tale of protein S and C4b-binding protein, a story of affection. Thromb Haemost. 2007; 98(1):90–6.

Eldor A, Durst R, Hy-Am E, Goldfarb A, Gillis S, Rachmilewitz EA et al. A chronic hypercoagulable state in patients with beta-thalassaemia is already present in childhood. Br J Haematol. 1999; 107(4):739–46.

Trinchero A, Marchetti M, Giaccherini C, Tartari CJ, Russo L, Falanga A. Platelet haemostatic properties in beta-thalassemia: the effect of blood transfusion. Blood Transfus. 2017; 15(5):413–421.

Borgna-Pignatti C, Rugolotto S, De Stefano P, Zhao H, Cappellini MD, Del Vecchio GC et al. Survival and complications in patients with thalassemia major treated with transfusion and deferoxamine. Hematologica. 2004; 89(10):1187–93.

Anderson JA, Hogg KE, Weitz JI. Hypercoagulable States. In: Hoffman R, Benz EJ, Silberstein LE, Heslop H, Weitz J, Anastasi J. (eds.) Hematology Basic Principles and Practice. 7th ed. New York: Elsevier; 2017: 2076–2087.


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