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Thrombin Activable Fibrinolysis Inhibitor in Beta Thalassemia
Aruna Chhikara1, Sunita Sharma2, Jagdish Chandra3
1Department of Pathology, Lady Hardinge Medical College, New Delhi, India. arunachhikara123@gmail.com.
Insights
Children with beta-thalassemia major show higher Thrombin activable fibrinolysis inhibitor (TAFI) levels, indicating an independent role in hypercoagulability. Early TAFI measurement may help detect coagulopathy and monitor thrombosis risk.
Area of Science:
- Hematology
- Pediatric Medicine
- Coagulation Disorders
Background:
- Beta-thalassemia major (TM) is a genetic blood disorder requiring frequent transfusions.
- TM patients often exhibit dysregulation of coagulation and fibrinolysis.
- Subclinical activation of hemostasis is suspected in TM.
Purpose of the Study:
- To investigate plasma levels of Thrombin activable fibrinolysis inhibitor (TAFI) in pediatric TM patients.
- To explore the correlation of TAFI with other coagulation parameters and clinical factors in TM.
Main Methods:
- Plasma TAFI levels were measured in 50 children with TM (age 1.4-17 years).
- Coagulation parameters including PT, aPTT, fibrinogen, D-dimer, Protein C, Protein S, antithrombin, t-PA, and PAI-1 were assessed.
- TAFI levels were compared between TM patients and a control group.
Main Results:
- TAFI levels were significantly higher in TM patients compared to controls (17.24 ± 4.05 ng/ml vs. 15.01 ± 3.28 ng/ml, p=0.003).
- Prolonged PT and aPTT were observed in 18% and 30% of cases, respectively.
- Reduced Protein C and Protein S were noted in 50% and 54% of cases, with significantly higher t-PA levels.
Conclusions:
- Elevated TAFI levels in TM patients suggest its independent contribution to a hypercoagulable state.
- TAFI may link coagulation and fibrinolysis, inhibiting fibrinolysis and promoting thrombosis risk.
- Measuring TAFI could aid in early detection of coagulopathy and thrombosis monitoring in TM.
Objectives:
To study plasma levels of Thrombin activable fibrinolysis inhibitor (TAFI) in children with β-thalassemia major.
Methods:
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.
Results:
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 ± 4.05 ng/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.
Conclusions:
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.
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