Highlights
- •Transfusion dependent thalassemia patients have a hypercoagulable status compared to age matched reference range.
- •Annual blood transfusion requirement was found to have a negative correlation with the hypercoagulable status.
- •Screening thalassemia patients with TEG may help to identify high risk thrombotic group.
Abstract
Background
Thromboembolic events are rare but one of the fatal complications in thalassemia.
Assessment of the hypercoagulable state is not done regularly, and we have assessed
the utility of Thromboelastography (TEG) for monitoring the activation of the coagulation
pathway in patients with thalassemia.
Methodology
A prospective single-center cohort study was conducted in a tertiary care set-up.
Transfusion Dependent Thalassemia patients registered with the pediatric unit were
screened for hypercoagulability using TEG during six months of the study period and
followed up for three years for the development of thromboembolic events. Patient
demographics, history of splenectomy, Serum ferritin levels and annual red cell transfusion
requirement (mL/kg/year) were assessed. TEG parameters used were R time, K time, alpha
angle, Maximum amplitude, Clot index, and Lysis 30. The thrombin generation test (V
Curve) obtained from the first-degree derivate of the TEG velocity curve was also
used for analysis.
Results
A total of 34 patients were recruited during the six months study period with an average
age of 10.6 years ( ± 5.47). The average pre-transfusion hemoglobin level and the
volume of packed red cells received were 7.24 g/dL and 152.82 mL/kg/year respectively.
The TEG tracing was suggestive of a hypercoagulable state in 58.82% of patients. The
mean values of angle (70.74), MA (64.16), CI (2.65) and TG (774.43) in TDT patients
compared to age matched reference range (62.81, 57.99, 0.8, 577.83 respectively) was
suggestive of prothrombotic changes. Annual blood transfusion requirement was negatively
correlated with hypercoagulable status (‐0.344, CI= -0.68 to 0.08). One out of 34
patients developed corona radiata infarct (with annual blood requirement; 112.7 mL/kg/Year).
The risk to develop a hypercoagulable state appeared to be higher when the volume
of RBCs transfused was less than 154 mL/kg/Year.
Conclusion
TDT patients are at risk of developing thromboembolism, and screening with TEG may
be useful to identify those at high risk.
Keywords
Abbreviations:
TEG (Thromboelastography), TDT (Transfusion Dependent Thalasemia), NTDT (Non Transfusion Dependent Thalassemia), TE (Thromboembolic Events), TM (Thalassemia Major), TI (Thalassemia Intermedia), MA (Maximum Amplitude), CI (Clot Index), MRTG (Maximum Rate of Thrombus Generation), TMRTG (Time to Maximum Rate of Thrombus Generation), TG (Thrombus Generated)To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Transfusion and Apheresis ScienceAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Coagulation defects in thalassemic patients.Pediatr Neonatol. 2017; 58: 421-424https://doi.org/10.1016/j.pedneo.2016.07.009
- Venous thromboembolism and hypercoagulability in splenectomized patients with thalassaemia intermedia.Br J Haematol. 2000; 111: 467-473
- A chronic hypercoagulable state in patients with β-thalassaemia major is already present in childhood.Br J Haematol. 1999; 107: 739-746
- The hypercoagulable state in thalassemia.Blood. 2002; 99: 36-43
- Overview on practices in thalassemia intermedia management aiming for lowering complication rates across a region of endemicity: the optimal care study.Blood. 2010; 115: 1886-1892
- Thalassemia and hypercoagulability.Blood Rev. 2008; 22: 283-292
- Hypercoagulability in β-thalassemia: a status quo.Expert Rev Hematol. 2012; 5: 505-512
- Hypercoagulability and vascular disease.Hematol Oncol Clin N Am. 2018; 32: 237-245
- Coagulopathy in beta-thalassemia: current understanding and future perspectives.Mediterr J Hematol Infect Dis. 2009; 1e2009029
- Thalassemia and hypercoagulability.Blood Rev. 2008; 22: 283-292
- Hypercoagulability in non-transfusion-dependent thalassemia.Blood Rev. 2012; 26: S20-S23https://doi.org/10.1016/S0268-960X(12)70007-3
- Hypercoagulable state as demonstrated by thromboelastometry in hemoglobin E/beta-thalassemia patients: association with clinical severity and splenectomy status.Thromb Res. 2016; 140: 125-131https://doi.org/10.1016/j.thromres.2016.02.014
- Global assays of hemostasis in the diagnostics of hypercoagulation and evaluation of thrombosis risk.Thromb J. 2015; 13
- Hypercoagulability in splenectomized thalassemic patients detected by whole-blood thromboelastometry, but not by thrombin generation in platelet-poor plasma.Haematologica. 2009; 94: 1520-1527
- Laboratory tests for coagulation system monitoring in a patient with β-thalassemia.Int J Hematol. 2014; 99: 588-596
- Thromboelastometry profile in children with beta-thalassemia.Int J Hematol. 2014; 99: 407-412
- Age- and gender-related reference ranges for thromboelastography from a healthy Indian population.Int J Lab Hematol. 2020; 42: 180-189
- Thromboelastograph: a prognostic marker in sepsis with organ dysfunction without overt bleeding.J Crit Care. 2021; 65: 177-183
- Consecutive thrombelastography clot strength profiles in patients with severe sepsis and their association with 28-day mortality: a prospective study.J Crit Care. 2013; 28: 317.e1-317.e11
- Thalassemia and venous thromboembolism.Mediterr J Hematol Infect Dis. 2011; 3e2011025
- Role of red blood cells “annexin V” and platelets “P-selectin” in patients with thalassemia.Hematol Oncol Stem Cell Ther. 2019; 12: 15-18
- The utility of thromboelastography in inherited and acquired bleeding disorders.Br J Haematol. 2016; 174: 503-514
- Fibrinolysis shutdown in COVID-19: clinical manifestations, molecular mechanisms, and therapeutic implications.J AM Coll Surg. 2021; 232: 995-1003
- Thalassemia trait and arterial thromboembolic events: a systematic review and a meta-analysis of the literature.J Thromb Haemost. 2011; 9: 917-921
Article Info
Publication History
Published online: October 18, 2022
Accepted:
October 14,
2022
Received in revised form:
October 10,
2022
Received:
April 4,
2022
Publication stage
In Press Journal Pre-ProofIdentification
Copyright
© 2022 Elsevier Ltd. All rights reserved.
