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

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 β-thalassemia, hemoglobin does not produce enough beta protein. According to mild to severe effects on the body, β-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 β-thalassemia major and their association to the hypercoagulable state. Seventy patients with β-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 βthalassemia major patients in comparison with control subjects (p-value <0.001). Mean of fibrinogen level (2.42) g/l was significantly decreased in β-thalassemia major patients while the mean of D dimer level (0.43) μg/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.

Protein C and S levels in patients with Thalassemia intermedia

“…One of the most recent complications in β-thalassemia patients is hypercoagulability, leading to severe morbidity and mortality [ 12 ]. This complication is not confined to β-thalassemia intermedia patients; activation of many hemostatic systems has also been demonstrated in persons with thalassemia major [ 12 – 14 ].…”

“…One of the most recent complications in β-thalassemia patients is hypercoagulability, leading to severe morbidity and mortality [ 12 ]. This complication is not confined to β-thalassemia intermedia patients; activation of many hemostatic systems has also been demonstrated in persons with thalassemia major [ 12 – 14 ]. Even though transfusion and splenectomy ignorance are by far the most common causes of thalassemia, plenty of other causal variables have been proposed [ 15 , 16 ].…”

Natural Anticoagulant Protein Levels in Patients With Beta-Thalassemia Major: A Case-Control Study

“…However, some studies have reported conflicting results. Hadi et al reported that one of their patients with β-TM had a PC level of 140% [4]. In addition, although Eldor et al reported a significant decrease in PC and PS levels, but the levels of AT were within the normal reference range [19].…”

“…This imbalance between α and β chains leads to an excess of α chains and subsequently early destruction of the erythrocytes, resulting in anemia [1][2][3]. The estimated annual prevalence of symptomatic patients with β-thalassemia worldwide is 100,000 [3,4]. In Iran, the estimated incidence of thalassemia is around 3.6%, making the country one of the regions with a high prevalence of thalassemia [2].…”

“…Inflammation is a contributing factor that may result in a reduction in the natural anticoagulant of the human body, and it is better to take this into account in patients with thalassemia as thalassemia is correlated with the presence of inflammation [14][15][16][17]. Several studies have investigated the levels of these natural anticoagulants in patients with β-thalassemia and have found lower levels of them compared to the control group (healthy individuals) [2,4,5,8,14,18]. However, some studies have reported conflicting results.…”