Abstract Number: PB0744
Meeting: ISTH 2020 Congress
Background: Thalassemia is an inherited blood disorder that is requiring lifelong transfusion treatment and is associated with a high incidence of thrombosis.
Aims: To investigate changes in the activity of natural anticoagulants in β-thalassemia patients.
Methods: A total of 127 patients with β-thalassemia major (β-TM) and β-thalassemia intermedia (β-TI) were studied for clinical evidence of thrombosis and were compared with 110 age and sex-matched healthy control. Protein C, S and antithrombin III (AT III) along with routine laboratory tests, serum levels of D-dimer and ferritin were measured. Patients with liver disease especially chronic hepatitis were excluded. The statistical analysis was performed using SPSS Software Version 23.0, chi-square, test-t and Pearson tests with a 95% confidence interval.
Results: The patient and control participant characteristics are summarized in Table 1. Of the 127 patients, 114 patients (89.5%) were splenectomized. 7 patients (5.5%) had a history of thrombosis and all were splenectomized. This group in comparison with Patients without thrombosis had lower Pro C, Pro S (p < 0.005). Concomitant reductions in Pro C, Pro S and AT III were observed in β-TM and β-TI than in controls (p < 0.005). Protein C decreased in 61% and 62% and protein S in 63% and 71% of β-TM and β-TI respectively. However, AT III decreased 13.4% and 32.7% of β-TM and β-TI (Table 2). When we compared Protein C, S and AT III between β-TM and β-TI only AT III showed a significant difference (P=0.001). PC and PS were reduced (p=0.001 and p=0.006) in nonsplenectomized patients compared with splenectomized patients.
Conclusions: Protein C, S and AT III significantly decreased in thalassemia patients compared to the control. These deficiencies of anticoagulant proteins might be contributing to chronic hypercoagulability in these patients. Thrombosis was seen in 7 (5.5%) patients and they showed lower protein C and S than patients without thrombosis.
|Subjects||N (%)||Age Median (Range)||Sex M/F||Splenectomy||Thrombophilia n(%)||Median Serum ferritin (µg/L)|
[Table1 Baseline characteristics in patients and controls]
|Variable (%)||β-TM (N = 64)||β-TI (N = 63)||Healthy control (N = 110)||Patients With Thrombosis (N = 7)|
[Table2 Level of Coagulation Inhibitors in Patients and Controls, Data are shown as median (range)]
To cite this abstract in AMA style:Mohsenian S, Zahedpanah M, Azarkeivan A, Seidi Zadeh O. Hypercoagulability State in Iranian Patients with β-Thalassemia [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/hypercoagulability-state-in-iranian-patients-with-%ce%b2-thalassemia/. Accessed November 30, 2021.
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