Abstract
Sickle cell disease (SCD) is associated with a significant hypercoagulable state and several hemostatic anomalies have been identified in this disease state. Of interest, SCD patients can become iron overloaded after transfusion, and iron can enhance fibrinogen as a substrate for thrombin, resulting in thrombi that commence coagulation quickly and form rapidly. We hypothesized that SCD patients would display hypercoagulable plasma coagulation kinetics and an iron enhancement of coagulation. After obtaining IRB approval, we assessed coagulation kinetics and iron enhancement with viscoelastic methods in archived, citrated plasma obtained from ambulatory or hospitalized SCD patients (n = 20). All SCD patients had plasmatic hypercoagulability, and 65 % were positive for iron enhancement of coagulation. In conclusion, continuing investigation correlating such viscoelastic data with clinical symptoms may provide insight into the role played by iron in the setting of SCD, including complications such as vaso-occlusive crisis.
Similar content being viewed by others
References
Pakbaz Z, Wun T (2014) Role of the hemostatic system on sickle cell disease: pathophysiology and potential therapeutics. Hematol Oncol Clin N Am 28:355–374
Shah S, Thornburg C, Telen MJ, Ortel TL (2012) Characterization of the hypercoagulable state in patients with sickle cell disease. Thromb Res 130:e241–e245
Porter J, Garbowski M (2013) Consequences and management of iron overload in sickle cell disease. Hematology Am Soc Hematol Educ Program 2013:447–456
Nielsen VG, Pretorius E (2014) Iron and carbon monoxide enhance coagulation and attenuate fibrinolysis by different mechanisms. Blood Coagul Fibrinolysis 25:695–702
Nielsen VG, Pretorius E (2014) Iron enhanced coagulation is attenuated by chelation: a thrombelastographic and ultrastructural analysis. Blood Coagul Fibrinolysis 25:845–850
Matika RW, Nielsen VG, Steinbrenner EB, Sussman AN, Madhrira M (2014) Hemodialysis patients have plasmatic hypercoagulability and decreased fibrinolytic vulnerability: role of carbon monoxide. ASAIO J 60:716–721
Ataga KI (2009) Novel therapies in sickle cell disease. Hematology Am Soc Hematol Educ Program. doi:10.1182/asheducation-2009.1.54
Adams RJ (2000) Lessons from the stroke prevention trial in sickle cell anemia (STOP) study. J Child Neurol 15:344–349
Drasar E, Igbineweka N, Vasavda N, Free M, Awogbade M, Allman M, Mijovic A, Thein SL (2011) Blood transfusion usage among adults with sickle cell disease—a single institution experience over ten years. Br J Haematol 152:766–770
Darbari DS, Kple-Faget P, Kwagyan J, Rana S, Gordeuk VR, Castro O (2006) Circumstances of death in adult sickle cell disease patients. Am J Hematol 81:858–863
Giordano PC, Huisman W, Harteveld CL (2011) Iron depletion: an ameliorating factor for sickle cell disease? ISRN Hematol 2011:473152
Jordan L, Adams-Graves P, Kanter-Washko J, Oneal PA, Sasane M, Vekeman F, Bieri C, Magestro M, Marcellari A, Duh MS (2014) Multicenter COMPACT study of COMplications in patients with sickle cell disease and utilization of iron chelation therapy. Curr Med Res Opin 31:1–11
Acknowledgment
This investigation was supported by the Department of Anesthesiology, the University of Arizona College of Medicine and the Department of Medicine, Duke University.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Shah, N., Welsby, I.J., Fielder, M.A. et al. Sickle cell disease is associated with iron mediated hypercoagulability. J Thromb Thrombolysis 40, 182–185 (2015). https://doi.org/10.1007/s11239-015-1230-6
Published:
Issue Date:
DOI: https://doi.org/10.1007/s11239-015-1230-6