• 1
    Nordström M, Lindblad B, Bergqvist D, Kjellstrom T. A prospective study of the incidence of deep-vein thrombosis within a defined urban population. J Intern Med 1992; 232: 15560.
  • 2
    Anderson FA Jr, Wheeler HB, Goldberg RJ, Hosmer DW, Patwardhan NA, Jovanovic B, Forcier A, Dalen JE. A population-based perspective of the hospital incidence and case-fatality rates of deep vein thrombosis and pulmonary embolism. The Worcester DVT Study. Arch Intern Med 1991; 151: 9338.
  • 3
    Koster T, Briët E, Van Der Meer FJ, Colly LP, Trienekens PH, Poort SR, Reitsma PH, Vandenbroucke JP. Protein C deficiency in a controlled series of unselected outpatients: an infrequent but clear risk factor for venous thrombosis (Leiden Thrombophilia Study). Blood 1995; 85: 275661.
  • 4
    Simmonds RE, Ireland H, Lane DA, Zoller B, Garcia de Frutos P, Dahlback B. Clarification of the risk for venous thrombosis associated with hereditary protein S deficiency by investigation of a large kindred with a characterized gene defect. Ann Intern Med 1998; 128: 814.
  • 5
    Bertina RM, Koeleman BP, Koster T, Rosendaal FR, Dirven RJ, De Ronde H, Van Der Velden PA, Reitsma PH. Mutation in blood coagulation factor V associated with resistance to activated protein C. Nature 1994; 369: 647.
  • 6
    Stearns-Kurosawa DJ, Kurosawa S, Mollica JS, Ferrell GL, Esmon CT. The endothelial cell protein C receptor augments protein C activation by the thrombin–thrombomodulin complex. Proc Natl Acad Sci USA 1996; 93: 102126.
  • 7
    Taylor FB Jr, Peer GT, Lockhart MS, Ferrell G, Esmon CT. Endothelial cell protein C receptor plays an important role in protein C activation in vivo. Blood 2001; 97: 16858.
  • 8
    Xu J, Qu DF, Esmon NL, Esmon CT. Metalloproteolytic release of endothelial cell protein C receptor. J Biol Chem 2000; 275: 603844.
  • 9
    Regan LM, Stearns-Kurosawa DJ, Kurosawa S, Mollica J, Fukudome K, Esmon CT. The endothelial cell protein C receptor. Inhibition of activated protein C anticoagulant function without modulation of reaction with proteinase inhibitors. J Biol Chem 1996; 271: 17499503.
  • 10
    Hurtado V, Montes R, Gris JC, Bertolaccini ML, Alonso A, Martinez-Gonzalez MA, Khamashta MA, Fukudome K, Lane DA, Hermida J. Autoantibodies against EPCR are found in antiphospholipid syndrome and are a risk factor for fetal death. Blood 2004; 104: 136974.
  • 11
    Montes R, Hurtado V, Alonso A, Foco L, Zonzin P, Mannucci PM, Hermida J. Autoantibodies against the endothelial receptor of protein C are associated with acute myocardial infarction in young women. J Thromb Haemost 2005; 3: 14548.
  • 12
    Espana F, Vaya A, Mira Y, Medina P, Estelles A, Villa P, Falco C, Aznar J. Low level of circulating activated protein C is a risk factor for venous thromboembolism. Thromb Haemost 2001; 86: 136873.
  • 13
    Zorio E, Navarro S, Medina P, Estelles A, Osa A, Rueda J, Cubillo P, Aznar J, Espana F. Circulating activated protein C is reduced in young survivors of myocardial infarction and inversely correlates with the severity of coronary lesions. J Thromb Haemost 2006; 4: 15306.
  • 14
    Lavigne-Lissalde G, Cochery-Nouvellon E, Granier G, Quere I, Gris JC. Diffuse skin necrosis in a patient with an anti-endothelial cell protein C receptor autoantibody which blocks protein C activation. J Thromb Haemost 2005; 3: 4135.
  • 15
    Koster T, Rosendaal FR, De Ronde H, Briët E, Vandenbroucke JP, Bertina RM. Venous thrombosis due to poor anticoagulant response to activated protein C: Leiden Thrombophilia Study. Lancet 1993; 342: 15036.
  • 16
    Van Der Meer FJ, Koster T, Vandenbroucke JP, Briët E, Rosendaal FR. The Leiden Thrombophilia Study (LETS). Thromb Haemost 1997; 78: 6315.
  • 17
    De Groot PG, Lutters B, Derksen RHWM, Lisman T, Meijers JC, Rosendaal FR. Lupus anticoagulants and the risk of a first episode of deep venous thrombosis. J Thromb Haemost 2005; 3: 19937.
  • 18
    Declerck PJ, Mombaerts P, Holvoet P, De Mol M, Collen D. Fibrinolytic response and fibrin fragment D-dimer levels in patients with deep vein thrombosis. Thromb Haemost 1987; 58: 10249.
  • 19
    Uitte de Willige S, Van Marion V, Rosendaal FR, Vos HL, De Visser MC, Bertina RM. Haplotypes of the EPCR gene, plasma sEPCR levels and the risk of deep venous thrombosis. J Thromb Haemost 2004; 2: 130510.
  • 20
    Woolf B. On estimating the relation between blood group and disease. Am J Hum Genet 1955; 19: 251.
  • 21
    Stearns-Kurosawa DJ, Swindle K, D’Angelo A, Della Valle P, Fattorini A, Caron N, Grimaux M, Woodhams B, Kurosawa S. Plasma levels of endothelial protein C receptor respond to anticoagulant treatment. Blood 2002; 99: 52630.
  • 22
    Fischetti F, Durigutto P, Pellis V, Debeus A, Macor P, Bulla R, Bossi F, Ziller F, Sblattero D, Meroni P, Tedesco F. Thrombus formation induced by antibodies to beta2-glycoprotein I is complement dependent and requires a priming factor. Blood 2005; 106: 23406.