Abstract
The association of the severe form of thalassemia with complications of blood transfusion, iron overload, bony deformity and gall stone is well described. Currently, due to improvement in the way of managing thalassemia, most of thalassemia patients survive longer. However more new complications had been observed. Increased incidence of thromboembolism among thalassemia patients had triggered various studies done to determine hypercoagulable state among thalassemia patients. Several factors for the hypercoagulable state had been identified such as RBC membrane disruption, chronic platelet activation and defect in coagulation pathway. This study was carried out to determine the level of Protein C, protein S, free protein S and antithrombin among thalassemia patients and to compare the level of Protein C, protein S, free Protein S and antithrombin level between thalassemia patients and healthy control. This was a case control study done at Hospital Universiti Sains Malaysia. Thirty six thalassemia patients who came for regular blood transfusion and 20 healthy blood donors for normal control were recruited. Blood samples were collected and analyzed for Protein C, protein S, free protein S and antithrombin using ACL Elite Pro. The result showed mean Protein C and free protein S levels were significantly lower (54.8 ± 13.2% and 70.0 ± 12.1% respectively) in thalassemia patients compared to age-matched normal control (94.1 ± 16.3% and 99.8 ± 17.3% respectively), whereas mean total protein S (54.8 ± 13.2% and 94.1 ± 16.3%; respectively) and antithrombin (70.0 ± 12.1% and 99.8 ± 17.3%; respectively) level were similar. In conclusion there was a significantly decreased Protein C and free protein S in thalassemia patients which might suggests hypercoagulable state in thalassemia patients. Since there are a lot of similarities in finding from other studies, we believe that many more study to look for other parameters contributing to hypercoagulable state in thalassemia patients is needed.
Highlights
Thalassemia is a common hereditary blood disorders that results from genetic defects causing deficient synthesis of hemoglobin polypeptide chains [1]
The phenomenon was seen more common in thalassemia intermedia patients than in regularly transfused thalassemia major patients
We have revealed a significantly decreased Protein C and protein S in thalassemia major patients which might suggests hypercoagulable state in this group
Summary
Thalassemia is a common hereditary blood disorders that results from genetic defects causing deficient synthesis of hemoglobin polypeptide chains [1]. The association of the severe form of the disease with complication of blood transfusion, iron overload, bony deformity and gall stone is well described. There has been increasing reports of haemostatic derangement complication which significantly influence the morbidity and mortality of this disease [2,3,4]. Wide spectrum of haemostatic abnormalities ranges from subclinical derangement of hemostatic parameters to various thromboembolic events such as pulmonary embolism, deep vein thrombosis and portal vein thrombosis have been reported [4]. The phenomenon was seen more common in thalassemia intermedia patients than in regularly transfused thalassemia major patients. The prevalence is higher in splenectomized patients [4,5]
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
