Procoagulant activity in patients with sickle cell trait

Blood Coagulation & Fibrinolysis : an International Journal in Haemostasis and Thrombosis
A S LawrieMaxwell P Westerman

Abstract

Patients with sickle cell trait (STr) are usually considered to be asymptomatic. However, complications, including hypercoagulability, increased risk of venous thromboembolism and the exertional exercise syndrome with rhabdomyolysis and sudden death, have been described. The exact cause of these adverse events is unclear. We have investigated two patients, a set of monozygotic twins with STr, to establish their procoagulant activity status as a potential indicator of thrombotic risk. In-vivo thrombin generation was assessed by the measurement of prothrombin fragment 1 + 2 (F1 + 2) and thrombin-antithrombin complexes (TAT). D-dimer was used as a marker of fibrinolytic activity. The potential to generate thrombin was determined using an ex-vivo thrombin generation test (TGT). The impact of red blood cell (RBC)-derived microparticle shedding and RBC rheology were examined. TAT (>60 μg/l) and F1 + 2 (948 pmol/l) were markedly elevated in patient 2 but within the normal reference range in patient 1 (TAT = 2.5 μg/l; F1 + 2 = 138 pmol/l). D-dimer levels (0.9 mg/l FEU) were similarly elevated in both patients. TGT peak thrombin and endogenous thrombin potential (ETP) were elevated to similar degrees in both patients. Flow cytometric an...Continue Reading

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Citations

Jun 11, 2016·Blood Cells, Molecules & Diseases·Maxwell Westerman, John B Porter
Oct 25, 2016·Translational Research : the Journal of Laboratory and Clinical Medicine·Michael TarasevSumita Chakraborty
May 4, 2017·Case Reports in Emergency Medicine·Kamille AbdoolAdrian Alexander
Sep 16, 2017·Arteriosclerosis, Thrombosis, and Vascular Biology·Laura M RaffieldUNKNOWN NHLBI Trans-Omics for Precision Medicine (TOPMed) Consortium, Hematology & Hemostasis TOPMed Working Group*

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