Protein C and Protein S Levels in Î²-Thalassemia Major Patients in Erbil, Kurdistan Region

Tareefa Kakakhan Hadi; Nawsherwan Sadiq Mohammad; Saran Abdulqadir Nooruldin

doi:10.14715/cmb/2020.66.5.5

Issue

Vol. 66 No. 5: Issue 5

Issue Published : August 6, 2020

The undersigned hereby assign all rights, included but not limited to copyright, for this manuscript to CMB Association upon its submission for consideration to publication on Cellular and Molecular Biology. The rights assigned include, but are not limited to, the sole and exclusive rights to license, sell, subsequently assign, derive, distribute, display and reproduce this manuscript, in whole or in part, in any format, electronic or otherwise, including those in existence at the time this agreement was signed. The authors hereby warrant that they have not granted or assigned, and shall not grant or assign, the aforementioned rights to any other person, firm, organization, or other entity. All rights are automatically restored to authors if this manuscript is not accepted for publication.

Protein C and Protein S Levels in Î²-Thalassemia Major Patients in Erbil, Kurdistan Region

https://doi.org/10.14715/cmb/2020.66.5.5

Tareefa Kakakhan Hadi

Nawsherwan Sadiq Mohammad

Saran Abdulqadir Nooruldin

Corresponding Author(s) : Nawsherwan Sadiq Mohammad

rahim.abdulkarimi@yahoo.com

Cellular and Molecular Biology, Vol. 66 No. 5: Issue 5
Article Published : July 31, 2020

Abstract

Oxygen is transported in the blood through red blood cells and a protein called hemoglobin. The protein consists of two alpha and two beta chains. The lack of any of these chains is caused by the malfunction of the genes that produce them, and can lead to a genetic disease called thalassemia. In Î²-thalassemia, hemoglobin does not produce enough beta protein. According to mild to severe effects on the body, Î²-thalassemia is divided into three types minor, interstitial and major thalassemia. There are increasing risks for thrombosis complications in thalassemia major. The purpose of this study was to evaluate protein C and protein S levels in Î²-thalassemia major and their association to the hypercoagulable state. Seventy patients with Î²–thalassemia major and 35 apparently healthy subjects as a control group were investigated for protein C and protein S. The mean of protein C (71.31%) and protein S (34.3%) levels were significantly reduced in Î²- thalassemia major patients in comparison with control subjects (p-value <0.001). Mean of fibrinogen level (2.42) g/l was significantly decreased in Î²-thalassemia major patients while the mean of D dimer level (0.43) Î¼g/ml was significantly increased in comparison to control subjects (p-value 0.001). This study demonstrates a chronic hypercoagulable state in B- thalassemia major patients.

Keywords

Beta Thalassemia Major Protein C Protein S Hypercoagulable State and Thromboembolic event.

Hadi, T. K., Mohammad, N. S., & Nooruldin, S. A. (2020). Protein C and Protein S Levels in Î²-Thalassemia Major Patients in Erbil, Kurdistan Region. Cellular and Molecular Biology, 66(5), 25–28. https://doi.org/10.14715/cmb/2020.66.5.5

Download Citation

References

References
Giardina PJ, Rivella S. Thalassemia syndromes. In: Hoffman R, Benz EJ Jr, Silberstein LE, Heslop HE, Weitz JI, Anastasi J, eds. Hematology: Basic Principles and Practice. 6th ed. Philadelphia, PA: Elsevier Saunders 2013; chap 38.
Kamil KH, Mohammad NS. A Laboratory Study of Anemia in Children Aged 6 Months to 6 Years in Erbil City. Med J Babylon 2014; 11 (2): 274-284.
Galanello R, Origa R. Beta-thalassemia. Orphanet J Rare Dis 2010; 5(11).
Borgna-Pignatti C, Cappellini MD, De Stefano P, Del Vecchio GC, Forni GL, Gamberini MR, et al. Survival and complications in thalassemia. Ann N Y Acad Sci 2005; 1054: 40-7.
Raz S, Koren A, Dan O, Levin C. Executive function and neural activation in adults with beta-thalassemia major: an event-related potentials study. Ann NY Acad Sci 2016; 1386:16–29
Palta S, Saroa R, Palta A. Overview of the coagulation system. Indian J Anaesth 2014; 58(5):515–523
Esmon CT, Vigano-D'Angelo S, D'Angelo A, Comp PC. Anticoagulant proteins C and S.
Adv Exp Med Biol 1987; 214: pp. 47-54
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; 3:60-65.
Vineeta S, Biswas A, Bijender K, Renu S. Protein C and protein S: causative factor for developing a hemorrhagic infarct in a HbE/Beta thalassemia child. Indian J Pediatr 2010; 77 (3): 316-317.
Eldor A, Rachmilewitz EA. The hypercoagulable state in thalassemia. Blood 2002; 99: 36-43.
Visudhiphan S, Ketsa-Ard K, Tumliang S, Piankijagum A. Significance of blood coagulation and platelet profiles in relation to pulmonary thrombosis in beta-thalassemia/Hb E. Southeast Asian J Trop Med Public Health 1994; 25 (3): 449-56.
Singer ST, Kuypers FA, Styles L, Vichinsky EP, Foote D, Rosenfeld H. Pulmonary Hypertension in Thalassemia: Association with Platelet Activation and Hypercoagulable State. Am J Haematol 2006; 81 (9): 670–675.
Cappellini M, Cohen A, Eleftheriou A, Piga A, Porter J. Endocrine Complications in Thalassaemia Major. In: Guidelines for the Clinical Management of Thalassaemia 2000; TIF; 41-49.
Elbedewy TA, Elshweikh SA, Abd El-Naby AY, Elsheikh EA. Pulmonary hypertension in adult Egyptian patients with b-thalassemia major: correlation with natural anticoagulant levels. Tanta Med J 2015; 43:52-9.
Angchaisuksiri P, Atichartakarn V, Aryurachai K, Archararit N, Chuncharunee S, Tiraganjana A, Rattanasiri S. Hemostatic and thrombotic markers in patients with hemoglobin E/beta-thalassemia disease. Am J Hematol 2007; 82(11):1001-1004.
Rosnah B, Noor Halina MN, Shafini Y, Marini R, Rosline H, Amal Hayati H. The level of natural anticoagulants in transfusion dependent thalassemia patients in Kelantan, Northeastern Malaysia. J Hematol Thrombo Dis 2014; 2:140-144.
Shirahata A, Funahara Y, Opartkiattikul N, Fucharoen S, Laosombat V, Yamada K. Protein C and protein S deficiency in thalassemic patients. Southeast Asian J Trop Med Public Health. 1992; 23:65-73.
Huang Y, Long Y, Deng D, Liu Z, Liang H, Sun N, Xu Y, Lai Y, Cheng P. Alterations of anticoagulant proteins and soluble endothelial protein C receptor in thalassemia patients of Chinese origin. Thrombosis Res 2018, 172; 61-66.
Abosdera MM, Almasry AE, Abdel-Moneim ES. Alterations of anticoagulant proteins and soluble endothelial protein C receptor in thalassemia patients of Chinese origin. Thrombosis Res 2018; 172: 61-66.
Musallam KM, Taher AT, Karimi M, Rachmilewitz EA. Cerebral infarction in Beta-thalassemia intermedia: breaking the silence. Thromb Res. 2012; 130(5):695-702.
Bordbar M, Darvishzadeh R, Pazhouhandeh M, Kahrizi D. An overview of genome editing methods based on endonucleases. Modern Genetics J 2020; 15(2): 75-92.
Hoseini M, Sahmani M, Foroughi F, Khazaei Monfared Y, Azad M. Evaluating the Role of PTEN Promoter Methylation in Patients Predisposed to Hypercoagulable States via Methylation Specific PCR. Rep Biochem Mol Biol. 2019;7(2):223"229.

References

Giardina PJ, Rivella S. Thalassemia syndromes. In: Hoffman R, Benz EJ Jr, Silberstein LE, Heslop HE, Weitz JI, Anastasi J, eds. Hematology: Basic Principles and Practice. 6th ed. Philadelphia, PA: Elsevier Saunders 2013; chap 38.

Kamil KH, Mohammad NS. A Laboratory Study of Anemia in Children Aged 6 Months to 6 Years in Erbil City. Med J Babylon 2014; 11 (2): 274-284.

Galanello R, Origa R. Beta-thalassemia. Orphanet J Rare Dis 2010; 5(11).

Borgna-Pignatti C, Cappellini MD, De Stefano P, Del Vecchio GC, Forni GL, Gamberini MR, et al. Survival and complications in thalassemia. Ann N Y Acad Sci 2005; 1054: 40-7.

Raz S, Koren A, Dan O, Levin C. Executive function and neural activation in adults with beta-thalassemia major: an event-related potentials study. Ann NY Acad Sci 2016; 1386:16–29

Palta S, Saroa R, Palta A. Overview of the coagulation system. Indian J Anaesth 2014; 58(5):515–523

Esmon CT, Vigano-D'Angelo S, D'Angelo A, Comp PC. Anticoagulant proteins C and S.

Adv Exp Med Biol 1987; 214: pp. 47-54

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; 3:60-65.

Vineeta S, Biswas A, Bijender K, Renu S. Protein C and protein S: causative factor for developing a hemorrhagic infarct in a HbE/Beta thalassemia child. Indian J Pediatr 2010; 77 (3): 316-317.

Eldor A, Rachmilewitz EA. The hypercoagulable state in thalassemia. Blood 2002; 99: 36-43.

Visudhiphan S, Ketsa-Ard K, Tumliang S, Piankijagum A. Significance of blood coagulation and platelet profiles in relation to pulmonary thrombosis in beta-thalassemia/Hb E. Southeast Asian J Trop Med Public Health 1994; 25 (3): 449-56.

Singer ST, Kuypers FA, Styles L, Vichinsky EP, Foote D, Rosenfeld H. Pulmonary Hypertension in Thalassemia: Association with Platelet Activation and Hypercoagulable State. Am J Haematol 2006; 81 (9): 670–675.

Cappellini M, Cohen A, Eleftheriou A, Piga A, Porter J. Endocrine Complications in Thalassaemia Major. In: Guidelines for the Clinical Management of Thalassaemia 2000; TIF; 41-49.

Elbedewy TA, Elshweikh SA, Abd El-Naby AY, Elsheikh EA. Pulmonary hypertension in adult Egyptian patients with b-thalassemia major: correlation with natural anticoagulant levels. Tanta Med J 2015; 43:52-9.

Angchaisuksiri P, Atichartakarn V, Aryurachai K, Archararit N, Chuncharunee S, Tiraganjana A, Rattanasiri S. Hemostatic and thrombotic markers in patients with hemoglobin E/beta-thalassemia disease. Am J Hematol 2007; 82(11):1001-1004.

Rosnah B, Noor Halina MN, Shafini Y, Marini R, Rosline H, Amal Hayati H. The level of natural anticoagulants in transfusion dependent thalassemia patients in Kelantan, Northeastern Malaysia. J Hematol Thrombo Dis 2014; 2:140-144.

Shirahata A, Funahara Y, Opartkiattikul N, Fucharoen S, Laosombat V, Yamada K. Protein C and protein S deficiency in thalassemic patients. Southeast Asian J Trop Med Public Health. 1992; 23:65-73.

Huang Y, Long Y, Deng D, Liu Z, Liang H, Sun N, Xu Y, Lai Y, Cheng P. Alterations of anticoagulant proteins and soluble endothelial protein C receptor in thalassemia patients of Chinese origin. Thrombosis Res 2018, 172; 61-66.

Abosdera MM, Almasry AE, Abdel-Moneim ES. Alterations of anticoagulant proteins and soluble endothelial protein C receptor in thalassemia patients of Chinese origin. Thrombosis Res 2018; 172: 61-66.

Musallam KM, Taher AT, Karimi M, Rachmilewitz EA. Cerebral infarction in Beta-thalassemia intermedia: breaking the silence. Thromb Res. 2012; 130(5):695-702.

Bordbar M, Darvishzadeh R, Pazhouhandeh M, Kahrizi D. An overview of genome editing methods based on endonucleases. Modern Genetics J 2020; 15(2): 75-92.

Hoseini M, Sahmani M, Foroughi F, Khazaei Monfared Y, Azad M. Evaluating the Role of PTEN Promoter Methylation in Patients Predisposed to Hypercoagulable States via Methylation Specific PCR. Rep Biochem Mol Biol. 2019;7(2):223"229.

Author biographies is not available.