Protein Z in ß-Thalassemia Major Adult Patients and Risk of Thrombosis

Recent studies have suggested an association between protein Z (PZ) deficiency and thrombosis. Profound haemostatic changes have been observed in patients with ß-thalassemia major with a higher incidence than normal of thromboembolic events, so our present study was carried to asses the role of PZ in hypercoagulable state in ß-thalassemia major with other coagulation factors inhibitors (protein C and S) and to compare the splenectomized versus non–splenectomized patients regarding the risk of thrombosis. The study included 44 patients with ß-thalassemia major (22 splenectomized 22 non-splenectomized), their age ranged from 20- 32 years. All were recruited from hematology department of Kaser AL Aini hospital. Fifteen healthy subjects who were age and sex matched were included as controls. Complete blood picture, liver function tests, serum ferritin, PZ antigen, PC PS activity and FDPs were measured for all participants. Study showed significant reduction of PZ antigen together with reduced PC and PS activity in thalassemic patients compared to controls (p 0.001), FDPs was higher in both thalassemic groups compared with control group (p 0.001). The reduction of PZ antigen, PC PS activity was more significant in splenectomized patients compared to non-splenectomized patients together with higher FDPs (All p values 0.001). An inverse correlation between FDPs and PZ antigen, FDPs and PC PS activity were detected. Conclusion: Reduced PZ antigen together with PC and PS activity may be a contributing factor for hypercoagulable state in thalassemia major. Splenectomized patients are at higher risk of thrombosis.