Coagulation and Fibrinolysis Dysregulation in β-Thalassemia Major: Potential Impact of Splenectomy and Medications on Thrombotic Risk.

ObjectiveThe dysregulation of clotting factors in β-thalassemia major is not fully understood. This case-control study examines the impact of splenectomy and medications on clotting factor dysregulation.MethodsLab. tests of coagulation and fibrinolysis were performed.ResultsThis study included 60 β-thalassemia major patients of both sexes (7-35 years) and 20 age-matched controls. None of the participants had a previous thrombotic event. No difference existed between control and β-thalassemia groups in all tested parameters except for protein S (PS) which was 26.1% lower in β-thalassemia group compared to the control group (p = 0.0001) while D-Dimer, plasminogen activator inhibitor-1 (PAI-1) and platelet count showed an increase in their levels by 53.6%, 93.4% and 112.7%, respectively compared to the control (p = 0.004, p = 0.0001, p = 0.0001, respectively). Notably, a strong positive correlation existed between platelet count and PAI-1 (r = 0.669, p = 0.0001) in β-thalassemia group. Splenectomized patients had higher platelet count (+45.2%), PAI-1 (+98.1%), fibrinogen (+18.9%) and tPA (+197.2%) compared to the non-splenectomized group (all p < 0.05). No significant differences in PS, D-dimer, PT, INR, aPTT, fibrinogen, PC, TF, tPA or ADAMTS13 were found between β-thalassemia major patients taking and not taking aspirin. However, a higher platelet count (+37.1%) and PAI-1 level (+58.9%), along with a lower vWF level (-25.6%), were observed between these two groups (all p < 0.05).ConclusionElevation in PAI-1 and platelet count in splenectomized β-thalassemia major could increase the risk of having a thrombotic event. Medications may have significant interactions with blood coagulation in β-thalassemia.