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
References
Sep 24, 1987·The New England Journal of Medicine·J A KarkC J Ruehle
Jan 1, 1985·The Journal of Clinical Investigation·P F FranckB Roelofsen
Sep 1, 1980·The Journal of Clinical Investigation·N MohandasS B Shohet
Apr 1, 1984·British Journal of Haematology·M P WestermanK Wu
Dec 21, 2000·JAMA : the Journal of the American Medical Association
Feb 9, 2002·American Journal of Hematology·M P WestermanP Williamson
Mar 1, 2002·Muscle & Nerve·Jason D WarrenPhilip D Thompson
Jul 11, 2003·Pathophysiology of Haemostasis and Thrombosis·H Coenraad HemkerSuzette Béguin
Nov 20, 2004·Journal of Thrombosis and Haemostasis : JTH·R Luddington, T Baglin
Mar 19, 2005·Critical Care : the Official Journal of the Critical Care Forum·Ana L Huerta-AlardínPaul E Marik
Apr 6, 2007·Blood·Harland AustinW Craig Hooper
Oct 10, 2007·Journal of Sport Rehabilitation·E Randy Eichner
Apr 22, 2008·British Journal of Haematology·Maxwell WestermanJohn Porter
Oct 10, 2009·Haematologica·Eduard J van BeersUNKNOWN CURAMA study group
Dec 1, 2009·Journal of Thrombosis and Haemostasis : JTH·T Baglin
Aug 25, 2010·American Journal of Medical Genetics. Part a·Hamid Reza RazzaghianJan P Dumanski
Citations
Mar 11, 2015·British Journal of Haematology·Nigel S KeyVimal K Derebail
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*