Thrombin Activable Fibrinolysis Inhibitor in Beta Thalassemia.

Abstract

To study plasma levels of Thrombin activable fibrinolysis inhibitor (TAFI) in children with β-thalassemia major. Fifty β-thalassemia major patients, 1.4 to 17 y of age, with number of transfusions received varying from 21 to 162 were selected at random and complete blood count (CBC), coagulation parameters [Prothrombin time (PT), Activated partial thromboplastin time (aPTT), fibrinogen, D-dimer, protein C, protein S, antithrombin, Tissue plasminogen activator (t-PA), Plasminogen activator inhibitor (PAI-1)] and TAFI were performed. PT and aPTT were prolonged in 18% and 30% of cases respectively. Reduced activity of Protein C (PC) was observed in 50% of cases and Protein S (PS) was reduced in 54% of cases. t-PA levels were significantly higher in cases. TAFI levels were 17.24±4.05ng/ml which were significantly higher than the control group (15.01±3.28; p=0.003) No significant correlation of TAFI was observed with Hb, platelet counts, liver enzymes, serum ferritin, PC, PS, D-dimer, t-PA or PAI-1. There is an ongoing subclinical activation of coagulation cascade and fibrinolytic system in thalassemia major (TM) patients. Higher levels of TAFI in the present study with no significant correlation with other parameters were noted, thus pointing out to its independent role in contribution to hypercoagulable state in thalassemia. TAFI serves as a link between two limbs of hemostasis, with its higher levels promoting inhibition of fibrinolytic system and thus promoting a hypercoagulable state. Performing TAFI levels in thalassemic patients could help to detect the early coagulopathy in these patients and hence these patients can be closely monitored for any evidence of thrombosis.