• 1
    Morawitz P. Die Chemie der Blutgerinnung. Ergeb Physiol 1905; 4: 307422.
  • 2
    Patek AJ, Stetson RP. Hemophilia. I. The abnormal coagulation of the blood and its relation to the blood platelets. J Clin Invest 1936; 15: 53142.
  • 3
    Dam H. The antihaemorrhagic vitamin of the chick. Biochem J 1935; 29: 127385.
  • 4
    Owren PA. Parahaemophilia. Haemorrhagic diathesis due to absence of a previously unknown clotting factor. Lancet 1947; 1: 4468.
  • 5
    Alexander B, Goldstein R, Landwehr G, Cook CD. Congenital SPCA deficiency: a hitherto unrecognized coagulation defect with hemorrhage rectified by serum and serum fractions. J Clin Invest 1951; 30: 596608.
  • 6
    Pavlovsky A. Contribution to the pathogenesis of hemophilia. Blood 1947; 2: 18591.
  • 7
    Aggeler PM, White SG, Glendening MB, Page EW, Leake TB, Bates G. Plasma thromboplastin component (PTC) deficiency: a new disease resembling hemophilia. Proc Soc Exp Biol Med 1952; 79: 6924.
  • 8
    Schulman I, Smith CH. Hemorrhagic disease in an infant due to deficiency of a previously undescribed clotting factor. Blood 1952; 7: 794807.
  • 9
    Biggs R, Douglas AS, Macfarlane RG, Dacie JV, Pitney WR, Mersky C, O’Brien JR. Christmas disease: a condition previously mistaken for haemophilia. Br Med J 1952; II: 137882.
  • 10
    Telfer TP, Denson KW, Wright DR. A ‘new’ coagulation defect. Br J Haematol 1956; 2: 30816.
  • 11
    Hougie C, Barrow EM, Graham JB. Stuart clotting defect. I. Segregation of an hereditary hemorrhagic state from the heterogeneous group heretofore called “stable factor” (SPCA, proconvertin, factor VII) deficiency. J Clin Invest 1957; 36: 48596.
  • 12
    Rosenthal RL, Dreskin OH, Rosenthal N. New hemophilia-like disease caused by deficiency of a third plasma thromboplastin factor. Proc Soc Exp Biol Med 1953; 82: 1714.
  • 13
    Duckert F, Jung E, Shmerling DH. A hitherto undescribed congenital haemorrhagic diathesis probably due to fibrin stabilizing factor deficiency. Thromb Diath Haemorrh 1960; 5: 17986.
  • 14
    Ratnoff OD, Colopy JE. A familiar hemorrhagic trait associated with a deficiency of a clot-promoting fraction of plasma. J Clin Invest 1955; 34: 60213.
  • 15
    Hathaway WE, Belhasen LP, Hathaway HS. Evidence for a plasma thromboplastin factor. I. Case report, coagulation studies and physicochemical properties. Blood 1965; 26: 52132.
  • 16
    Saito H, Ratnoff OD, Waldmann R, Abraham JP. Fitzgerald trait. Deficiency of a hitherto unrecognized agent, Fitzgerald factor, participating in surface-mediated reactions of clotting, fibrinolysis, generation of kinins, and the property of diluted plasma enhancing vascular permeability (PF/Dil). J Clin Invest 1975; 55: 10829.
  • 17
    Howell WH, Holt E. Two new factors in blood coagulation- heparin and proantithrombin. Am J Physiol 1918; 47: 32841.
  • 18
    Egeberg O. Inherited antithrombin deficiency causing thrombophilia. Thromb Diath Haemorrh 1965; 13: 51630.
  • 19
    Stenflo J. A new vitamin K-dependent protein: purification from bovine plasma and preliminary characterization. J Biol Chem 1976; 251: 35563.
  • 20
    DiScipio RG, Hermodson MA, Yates SG, Davie EW. A comparison of human prothrombin, factor IX (Christmas factor), factor X (Stuart factor), and protein S. Biochemistry 1977; 16: 698706.
  • 21
    Griffin JH, Evatt B, Zimmerman TS, Kleiss AJ, Wideman C. Deficiency of protein C in congenital thrombotic disease. J Clin Invest 1981; 68: 13703.
  • 22
    Comp PC, Nixon RR, Cooper MR, Esmon CT. Familiar protein S deficiency is associated with recurrent thrombosis. J Clin Invest 1984; 74: 20828.
  • 23
    Dahlbäck B, Carlsson M, Svensson PJ. Familial thrombophilia due to a previously unrecognized mechanism characterized by poor anticoagulant response to activated protein C: prediction of a cofactor to activated protein C. Proc Natl Acad Sci USA 1993; 90: 10048.
  • 24
    Bertina RM, Koeleman BPC, 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 (Lond) 1994; 369: 647.
  • 25
    Quick AJ, Stanley-Brown M, Bancroft FW. A study of the coagulation defect in hemophilia and in jaundice. Am J Med Sci 1935; 190: 50111.
  • 26
    Langdell RD, Wagner RH, Brinkhous KM. Effect of anti-hemophilic factor on one-stage clotting tests: a presumptive test for hemophilia and a simple one-stage anti-hemophilic factor assay procedure. J Lab Clin Med 1953; 41: 63747.
  • 27
    Davie EW, Ratnoff OD. Waterfall sequence of for intrinsic blood coagulation. Science 1964; 145: 13102.
  • 28
    Macfarlane RG. An enzyme cascade in the blood coagulation mechanism, and its function as a biochemical amplifier. Nature (Lond) 1964; 202: 4989.
  • 29
    Rapaport SI, Shiffman S, Patch MJ, Ames SB. The importance of activation of anti-haemophilic globulin and proaccelerin by traces of thrombin in the generation of intrinsic prothrombinase activity. Blood 1963; 21: 22136.
  • 30
    Nesheim ME, Taswell JB, Mann KG. The contribution of bovine factor V and factor Va to the activity of prothrombinase. J Biol Chem 1979; 254: 1095262.
  • 31
    Osterud B, Rapaport S. Activation of factor IX by the reaction product of tissue factor and factor VII: additional pathway for initiating blood coagulation. Proc Natl Acad Sci USA 1977; 74: 52604.
  • 32
    Gailani D, Broze GJ Jr. Factor XI activation in a revised model of blood coagulation. Science 1991; 253: 90912.
  • 33
    Naito K, Fujikawa K. Activation of human blood coagulation factor XI independent of factor XII: Factor XI is activated by thrombin and factor XIa in the presence of negatively charged surfaces. J Biol Chem 1991; 266: 73538.
  • 34
    Saito H. Studies on Fletcher trait and Fitzgerald trait- A rare chance to disclose body’s defense reactions against injury. Thromb Haemost 2010; 104: 86774.
  • 35
    von Willebrand EA. Fin. Uber hereditare Pseudohemophilie. Acta Med Scand 1931; 76: 52149.
  • 36
    Nilsson IM, Blombäck M, Jorpes E, Blombäck B, Johansson SA. von Willebrand’s disease and its correction with human plasma fraction 1-0. Acta Med Scand 1957; 159: 17988.
  • 37
    Zimmerman TS, Ratnoff OD, Powell AE. Immunologic differentiation of classic hemophilia (factor VIII deficiency) and von Willebrand’s disease, with observations on combined deficiencies of antihemophilic factor and proaccelerin (factor V) and on an acquired circulating anticoagulant against antihemophilic factor. J Clin Invest 1971; 50: 24454.
  • 38
    Abildgaard U. Highly purified antithrombin III with heparin cofactor activity prepared by disc electrophoresis. Scand J Clin Lab Invest 1968; 21: 8991.
  • 39
    Rosenberg RD, Damus PS. The purification and mechanism of action of human antithrombin-heparin cofactor. J Biol Chem 1973; 248: 6490505.
  • 40
    Esmon NL, Owen WG, Esmon CT. Isolation of a membrane-bound cofactor for thrombin-catalyzed activation of protein C. J Biol Chem 1982; 257: 85964.
  • 41
    Comp PC, Jacocks RM, Ferrell GL, Esmon CT. Activation of protein C in vivo. J Clin Invest 1982; 70: 12734.
  • 42
    Esmon CT. The protein C pathway. Chest 2003; 124: 26S32S.
  • 43
    Tollefsen DM, Majerus DW, Blank MK. Heparin cofactor II: purification and properties of a heparin-dependent inhibitor of thrombin in human plasma. J Biol Chem 1982; 257: 21629.
  • 44
    Broze GJ Jr, Miletich JP. Isolation of the tissue factor inhibitor produced by HepG2 hepatoma cells. Proc Natl Acad Sci USA 1987; 84: 188690.
  • 45
    Blombäck B, Blombäck M, Henschen A, Hessel B, Iwanaga S, Woods KR. N-terminal disulphide knot of human fibrinogen. Nature 1968; 218: 1304.
  • 46
    Doolittle RF, Watt KWK, Cottrell BA, Strong DD, Riley M. The amino acid sequence of the α-chain of human fibrinogen. Nature 1979; 280: 4648.
  • 47
    Kurachi K, Davie EW. Isolation and characterization of a cDNA coding for human factor IX. Proc Natl Acad Sci USA 1982; 79: 64614.
  • 48
    Choo KH, Gould KG, Rees DJ, Brownlee GG. Cloning of the gene for human anti-haemophilic factor IX. Nature 1982; 299: 17880.
  • 49
    Sadler JE, Shelton-Inloes BB, Sorace JM, Harlan JM, Titani K, Davie EW. Cloning and characterization of two cDNAs coding for human von Willebrand factor. Proc Natl Acad Sci USA 1985; 82: 63948.
  • 50
    Ginsberg D, Handin RI, Bonthron DT, Donlon TA, Bruns GA, Latt SA, Orkin SH. Human von Willebrand factor (vWF): isolation of complementary DNA (cDNA) clones and chromosomal localization. Science 1985; 228: 14016.
  • 51
    Jenny RJ, Pittman DD, Toole JJ, Kriz RW, Aldape RA, Hewick RM, Kaufman RJ, Mann KG. Complete cDNA and derived amino acid sequence of human factor V. Proc Natl Acad Sci USA 1987; 84: 484650.
  • 52
    Morrissey JH, Fakhrai H, Edgington TS. Molecular cloning of the cDNA for tissue factor, the cellular receptor for the initiation of the coagulation protease cascade. Cell 1987; 50: 12935.
  • 53
    Spicer EK, Horton R, Bloem L, Bach R, Williams KR, Guha A, Kraus J, Lin TC, Nemerson Y, Konigsberg WH. Isolation of cDNA clones coding for human tissue factor: primary structure of the protein and cDNA. Proc Natl Acad Sci USA 1987; 84: 514852.
  • 54
    Scarpati EM, Wen D, Broze JG Jr, Miletich JP, Flandermeyer RR, Siegel NR, Sadler JE. Human tissue factor: cDNA sequence and chromosome localization of the gene. Biochemistry 1987; 26: 52348.
  • 55
    Reitsma PH, Bernardi F, Doig RG, Gandrille S, Greengard JS, Ireland H, Krawczak M, Lind B, Long GL, Poort SR, Saito H, Sala N, Witt I, Cooper DN. Protein C deficiency: a database of mutations, 1995 update. Thromb Haemost 1995; 73: 87689.
  • 56
    Gandrille S, Borgel D, Sala N, Espinosa-Parrilla Y, Simmonds R, Rezende S, Lind B, Mannhalter C, Pabinger I, Reistma PH, Formstone C, Cooper DN, Saito H, Suzuki K, Bernardi F, Aiach M. Protein S deficiency: a database of mutations-summary of the first update. Thromb Haemost 2000; 84: 91834.
  • 57
    Rogaev EI, Grigorenko AP, Faskhutdinova G, Kittler ELW, Moliaka YK. Genotype analysis identifies the cause of the “Royal Disease”. Science 2009; 326: 817.
  • 58
    Yamamoto K, Tanimoto M, Emi N, Matsushita T, Takamatsu J, Saito H. Impaired secretion of the elongated mutant of protein C (protein C-Nagoya): molecular and cellular basis for hereditary protein C deficiency. J Clin Invest 1992; 90: 243946.
  • 59
    Matsushita T, Kojima T, Emi N, Takahashi I, Saito H. Impaired Human Tissue Factor-mediated Activity in Blood Clotting Factor VIInagoya (Arg304[RIGHTWARDS ARROW]Trp). J Biol Chem 1994; 269: 735563.
  • 60
    Levy GG, Nichols WC, Lian EC, Foround T, McClintick JN, McGee BM, Yang AY, Siemieniak DR, Stark KR, Gruppo R, Sarode R, Shurin SB, Chandrasekaran V, Stabler SP, Sabio H, Bouhassira EE, Upshaw JD Jr, Ginsberg D, Tsai H-M. Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura. Nature 2001; 413: 48894.
  • 61
    Fujikawa K, Suzuki H, McMullen B, Chung D. Purification of human von Willebrand factor-cleaving protease and its identification as a new member of the metalloproteinase family. Blood 2001; 98: 16626.
  • 62
    Gerritsen HE, Robles R, Lammale B, Furlan M. Partial amino acid sequence of purified von Willebrand factor-cleaving protease. Blood 2001; 98: 165461.
  • 63
    Nichols WC, Seligsohn U, Zivelin A, Terry VH, Hertel CE, Wheatley MA, Moussalli MJ, Hauri HP, Ciavarella N, Kaufman RJ, Ginsberg D. Mutations in the ER-Golgi intermediate compartment protein ERGIC-53 cause combined deficiency of coagulation factors V and VIII. Cell 1998; 93: 6170.
  • 64
    Zhang B, Cunningham MA, Nichols WC, Bernat JA, Seligsohn U, Pipe SW, McVey JH, Schulte-Overberg U, de Bosch NB, Ruiz-Saez A, White GC, Tuddenham EG, Kaufman RJ, Ginsberg D. Bleeding due to disruption of a cargo-specific ER-to-Golgi transport complex. Nat Genet 2003; 34: 2205.
  • 65
    Sun WY, Witte DP, Degen JL, Colbert MC, Burkart MC, Holmbäck K, Xiao Q, Bugge TH, Degen SJF. Prothrombin deficiency results in embryonic and neonatal lethality in mice. Proc Natl Acad Sci USA 1998; 95: 7597602.
  • 66
    Xue J, Wu Q, Westfield LA, Tuley EA, Lu D, Zhang Q, Shim K, Zheng X, Sadler JE. Incomplete embryonic lethality and fatal neonatal hemorrhage caused by prothrombin deficiency in mice. Proc Natl Acad Sci USA 1998; 95: 76037.
  • 67
    Cui J, O’Shea KS, Purkayastha A, Saunders TL, Ginsburg D. Fetal haemorrhage and incomplete block to embryogenesis in mice lacking coagulation factor V. Nature 1996; 384: 668.
  • 68
    Rosen ED, Chan JCY, Idusogie E, Clotman F, Vlasuk G, Luther T, Jalbert LR, Albrecht S, Zhong L, Lissens A, Schoonjans L, Moons L, Collen D, Castellino FJ, Carmeliet P. Mice lacking factor VII develop normally but suffer fatal perinatal bleeding. Nature 1997; 390: 2904.
  • 69
    Dewerchin M, Liang Z, Moons L, Carmeliet P, Castellino FJ, Collen D, Rosen ED. Blood coagulation factor X deficiency causes partial embryonic lethality and fatal neonatal bleeding in mice. Thromb Haemost 2000; 83: 18590.
  • 70
    Bugge TH, Xiao Q, Kombrinck KW, Flick MJ, Holmbäck K, Danton MJ, Colbert MC, Witte DP, Fujikawa K, Davie EW, Degen JL. Fatal embryonic bleeding events in mice lacking tissue factor, the cell-associated initiator of blood coagulation. Proc Natl Acad Sci USA 1996; 93: 625863.
  • 71
    Carmeliet P, Mackman N, Moons L, Luther T, Gressens P, van Vlaenderen I, Demunck H, Kasper M, Breier G, Evrard P, Müller M, Risau W, Edgington T, Collen D. Role of tissue factor in embryonic blood vessel development. Nature 1996; 383: 735.
  • 72
    Toomey JR, Kratzer KE, Lasky Nm, Stanton JJ, Broze GJ Jr. Targeted disruption of murine tissue factor gene results in embryonic lethality. Blood 1996; 88: 15837.
  • 73
    Bi L, Lawler AM, Antonarakis SE, High KA, Gearhart JD, Kazazian HH. Targeted disruption of the mouse factor VIII gene produces a model of haemophilia A. Nat Genet 1995; 10: 11921.
  • 74
    Lin H-F, Maeda N, Smithies O, Straight DL, Stafford DW. A coagulation factor IX-deficient mouse model for human hemophilia B. Blood 1997; 90: 39626.
  • 75
    Wang L, Zoppe M, Hackeng TM, Griffin JH, Lee K-F, Verma IM. A factor IX-deficient mouse model for hemophilia B gene therapy. Proc Natl Acad Sci USA 1997; 94: 115636.
  • 76
    Gailani D, Lasky NM, Broze GJ Jr. A murine model of factor XI deficiency. Blood Coagul Fibrinolysis 1997; 8: 13444.
  • 77
    Pauer HU, Renné T, Hemmerlein B, Legler T, Fritzlar S, Adham I, Müller-Esterl W, Emons G, Sancken U, Engel W, Burfeind P. Targeted deletion of murine coagulation factor XII gene- a model for contact phase activation in vivo. Thromb Haemost 2004; 92: 5038.
  • 78
    Lauer P, Metzner HJ, Zettlmeissl G, Li M, Smith AG, Lathe R, Dickneite G. Targeted inactivation of the mouse locus encoding coagulation factor XIII-A: hemostatic abnormalities in mutant mice and characterization of the coagulation deficit. Thromb Haemost 2002; 88: 96774.
  • 79
    Suh TT, Holmbäck K, Jensen NJ, Daugherty CC, Small K, Simon DI, Potter S, Degen JL. Resolution of spontaneous bleeding events but failure of pregnancy in fibrinogen-deficient mice. Genes Dev 1995; 9: 202033.
  • 80
    Ishiguro K, Kojima T, Kadomatsu K, Nakayama Y, Takagi A, Suzuki M, Takeda N, Ito M, Yamamoto K, Matsushita T, Kusugami K, Muramatsu T, Saito H. Complete antithrombin deficiency in mice results in embryonic lethality. J Clin Invest 2000; 106: 8738.
  • 81
    Jalbert LR, Rosen ED, Moons L, Chan JC, Carmeliet P, Collen D, Castellino FJ. Inactivation of the gene for anticoagulant protein C causes lethal perinatal consumption coagulopathy in mice. J Clin Invest 1998; 102: 14818.
  • 82
    Healy AM, Rayburn HB, Rosenberg RD, Weiler H. Absence of the blood-clotting regulator thrombomodulin causes embryonic lethality in mice before development of a functional cardiovascular system. Proc Natl Acad Sci USA 1995; 92: 8504.
  • 83
    Huang ZF, Higuchi D, Lasky N, Broze GJ Jr. Tissue factor pathway inhibitor gene disruption produces intrauterine lethality in mice. Blood 1997; 90: 94451.
  • 84
    Renné T, Pozgaiová M, Gruner S, Schuh K, Pauer HU, Burfeind P, Gailani D, Nieswandt B. Defective thrombus formation in mice lacking coagulation factor XII. J Exp Med 2005; 202: 27181.
  • 85
    Wang X, Smith PL, Hsu MY, Gailani D, Schumacher WA, Ogletree ML, Seiffert DA. Effects of factor XI deficiency on ferric chloride-induced vena cava thrombosis in mice. J Thromb Haemost 2006; 4: 19828.
  • 86
    Zhang H, Löwenberg EC, Crosby JR, MacLeod AR, Zhao C, Gao D, Black C, Revenko AS, Meijers JC, Stroes ES, Levi M, Monia BP. Inhibition of the intrinsic coagulation pathway factor XI by antisense oligonucleotides: a novel antithrombotic strategy with lowered bleeding risk. Blood 2010; 116: 468492.
  • 87
    Merkulov S, Zhang WM, Komar AA, Schmaier AH, Barnes E, Zhou Y, Lu X, Iwaki T, Catellino FJ, Luo G, McCrae KR. Deletion of murine kininogen gene 1 (mKng1) causes loss of plasma kininogen and delays thrombosis. Blood 2008; 111: 127481.
  • 88
    Svendsen L, Blombäck B, Blombäck M, Olsson PI. Synthetic chromogenic substrates for determination of trypsin, thrombin and thrombin-like enzymes. Thromb Res 1972; 1: 26778.
  • 89
    Zimmerman TS, Ratnoff OD, Littell AS. Detection of carriers of classic hemophilia using immunologic assay of antihemophilic factor (factor VIII). J Clin Invest 1971; 50: 2558.
  • 90
    Oberle I, Camerino G, Heilig R, Grunebaum L, Cazenave JP, Crapanzano C, Mannucci PM, Mandel JL. Genetic screening for hemophilia A (classic hemophilia) with a polymorphic DNA probe. N Engl J Med 1985; 312: 6826.
  • 91
    Antonarakis SE, Waber PG, Kittur SD, Patel AS, Kazazian HH Jr, Mellis MA, Counts RB, Stamatoyannopoulos G, Bowie EJ, Fass DN, Pittman DD, Wozney JM, Toole JJ. Hemophilia A detection of molecular defects and of carriers by DNA analysis. N Engl J Med 1985; 313: 8428.
  • 92
    Gitschier J, Lawn RM, Rotblat F, Goldman E, Tuddenham EGD. Antenatal diagnosis and carrier detection of haemophilia A using factor VIII gene probe. Lancet 1985; 1: 10934.
  • 93
    Kojima T, Tanimoto M, Kamiya T, Obata Y, Takahashi T, Ohno R, Kurachi K, Saito H. Possible absence of common polymorphisms in coagulation factor IX gene in Japanese. Blood 1987; 69: 34952.
  • 94
    Nossel HL, Younger LR, Wilner GD, Procupez T, Canfield RE, Butler VP Jr. Radioimmunoassay of human fibrinopeptide A. Proc Natl Acad Sci USA 1971; 68: 23503.
  • 95
    Lau HK, Rosenberg RD. The isolation and characterization of a specific antibody population directed against the thrombin antithrombin complex. J Biol Chem 1980; 255: 588593.
  • 96
    Lau HK, Rosenberg JS, Beeler DL, Rosenberg RD. The isolation and characterization of a specific antibody population directed against the prothrombin activation fragments F2 and F1+2. J Biol Chem 1979; 254: 875161.
  • 97
    Gaffney PJ, Lane DA, Kakkar VV, Brasher M. Characterisation of a soluble D dimer-E complex in crosslinked fibrin digests. Thromb Res 1975; 7: 8999.
  • 98
    Hemker HC, Veltkamp JJ, Loeliger EA. Kinetic aspects of the interaction of blood clotting enzymes. III. Demonstration of an inhibitor of prothrombin conversion in vitamin K deficiency. Thromb Diath Haemorrh 1968; 19: 34663.
  • 99
    Blanchard RA, Furie BC, Jorgensen M, Kruger SF, Furie B. Acquired vitamin K-dependent carboxylation deficiency in liver disease. N Engl J Med 1981; 305: 2428.
  • 100
    Blombäck M, Nilsson IM. Treatment of hemophilia A with human antihemophilic globulin. Acta Med Scand 1958; 161: 30121.
  • 101
    Kekwick RA, Wolf P. Concentrate of human antihaemophilic factor: its use in six cases of haemophilia. Lancet 1957; I: 64750.
  • 102
    Pool JG, Shannon AE. Production of high-potency concentrates of antihemophilic globulin in a closed-bag system. Assay in vitro and in vivo. N Engl J Med 1965; 273: 14437.
  • 103
    Rabiner SF, Telfer MC. Home Transfusion for Patients with Hemophilia A. N Engl J Med 1970; 283: 10115.
  • 104
    Davis KC, Horsburgh CR Jr, Hasiba U, Schocket AL, Kirkpatrick CH. Acquired immunodeficiency syndrome in a patient with hemophilia. Ann Intern Med 1983; 98: 2846.
  • 105
    White GC 2nd, McMillan CW, Kingdon HS, Shoemaker CB. Use of recombinant antihemophilic factor in the treatment of two patients with classic hemophilia. N Engl J Med 1989; 320: 16670.
  • 106
    Roth DA, Kessler CM, Pasi KJ, Rup B, Courter SG, Tubridy KL. Human recombinant factor IX: safety and efficacy studies in hemophilia B patients previously treated with plasma-derived factor IX concentrates. Blood 2001; 98: 36006.
  • 107
    Pipe SW. The promise and challenges of bioengineered recombinant clotting factors. J Thromb Haemost 2005; 3: 1692701.
  • 108
    Kurczynski EM, Penner JA. Activated prothrombin concentrate for patients with factor VIII inhibitors. N Engl J Med 1974; 291: 1647.
  • 109
    Lusher JM, Shapiro SS, Palascak JE, Rao AV, Levine PH, Blatt PM; the Hemophilia Study Group. Efficacy of prothrombin-complex concentrates in hemophiliacs with antibodies to factor VIII. A multicenter therapeutic trial. N Engl J Med 1980; 303: 4215.
  • 110
    Hedner U, Kisiel W. Use of human factor VIIa in the treatment of two hemophilic patients with high titer inhibitors. J Clin Invest 1983; 21: 183641.
  • 111
    Roberts HR, Monroe DM, White GC. The use of recombinant factor VIIa in the treatment of bleeding disorders. Blood 2004; 104: 385864.
  • 112
    Levi M, Levy JH, Andersen HF, Truloff D. Safety of recombinant activated factor VII in randomized clinical trials. N Engl J Med 2010; 363: 1791800.
  • 113
    Murray G. Heparin in thrombosis and embolism. Br J Surg 1939; 27: 56798.
  • 114
    Schofield FW. A brief account of a disease in cattle simulating hemorrhagic septicaemia due to feeding sweet clover. Can Vet Rec 1922; 3: 748.
  • 115
    Stahmann MA, Huebner CF, Link KP. Studies on the hemorrhagic sweet clover disease. V. Identification and synthesis of the hemorrhagic agent. J Biol Chem 1941; 138: 51327.
  • 116
    Bingham JB, Meyer OO, Pohle FJ. Studies on the hemorrhagic agent 3,3′-methylenebis (4-hydroxycoumarin). I. Its effect on the prothrombin and coagulation time of the blood of dogs and humans. Am J Med Sci 1941; 202: 56378.
  • 117
    Allen EV, Barker NW, Waugh JM. A preparation from spoiled sweet clover. [3-3′ methylene-bis-(4-hydroxycoumarin) which prolongs coagulation and prothrombin time of the blood: a clinical study. JAMA 1942; 120: 100915.
  • 118
    Esmon CT, Sadowski JA, Suttie JW. A new carboxylation reaction. The vitamin K-dependent incorporation of H14Co3 into prothrombin. J Biol Chem 1975; 250: 47448.
  • 119
    Whitlon DS, Sadowski JA, Suttie JW. Mechanism of coumarin action: significance of vitamin K epoxide reductase inhibition. Biochemisty 1978; 17: 13717.
  • 120
    The International Warfarin pharmacogenetics consortium. Estimation of the Warfarin dose with clinical and pharmacogenetic data. N Engl J Med 2009; 360: 75364.
  • 121
    Okamoto S, Hijikata A, Kikumoto R, Tonomura S, Hara H, Ninomiya K. Potent inhibition of thrombin by the newly synthesized arginine derivative No. 805. The importance of stereostructure of its hydrophobic carboxamide portion. Biochem Biophys Res Commun 1981; 101: 4406.
  • 122
    Connolly SJ, Ezekowitz MD, Yusuf S, Eikelboom J, Oldgren J, Parekh A, Pogue J, Reilly PA, Themeles E, Varrone J, Wang S, Alings M, Xavier D, Zhu J, Diaz R, Lewis BS, Darius H, Diener H-C, Joyner CD, Wallentin L; RE-LY Steering Committee and Investigators. Dabigatran versus warfarin in patients with atrial fibrillation. N Engl J Med 2009; 361: 113951.
  • 123
    Turpie AG, Bauer KA, Erksson BI, Lassen MR. Fondaparinux versus enoxaparin for the prevention of venous thromboembolism in major orthopedic surgery. A meta-analysis of 4 randomized double-blind studies. Arch Intern Med 2002; 162: 183340.
  • 124
    EINSTEIN Investigators, Bauersachs R, Berkowitz SD, Brenner B, Büller HR, Decousus H, Gallus AS, Lensing AW, Misselwitz F, Prins MH, Raskob GE, Segers A, Verhamme P, Wells P, Agnelli G, Bounameaux H, Cohen A, Davidson BL, Piovella F, Schellong S. Oral Rivaroxaban for symptomatic venous thromboembolism. N Engl J Med 2010; 363: 2499510.
  • 125
    Lassen MR, Gallus A, Raskob GE, Pineo G, Chen D, Ramirez LM; ADVANCE-3 Investigators. Apixaban versus enoxaparin for thromboprophylaxis after hip replacement. N Engl J Med 2010; 363: 248798.
  • 126
    Raskob G, Cohen AT, Eriksson BI, Puskas D, Shi M, Bocanegra T, Weitz JI. Oral direct factor Xa inhibition with edoxaban for thromboprophylaxis after elective total hip replacement. A randomized double-blind dose-response study. Thromb Haemost 2010; 104: 6429.
  • 127
    Kienast J, Juers M, Wiedermann CJ, Hoffmann JN, Ostermann H, Strauss R, Keinecke HO, Warren BL, Opal SM; KyberSept investigators. Treatment effects of high-dose antithrombin without concomitant heparin in patients with severe sepsis with or without disseminated intravascular coagulation. J Thromb Haemost 2006; 4: 907.
  • 128
    Bernard GR, Vincent JL, Laterre PF, LaRosa SP, Dhainaut JF, Lopez-Rodriguez A, Steingrub JS, Garber GE, Helterbrand JD, Ely EW, Fisher CJ Jr; Recombinant Human Activated Protein C Worldwide Evaluation in Severe Sepsis (PROWESS) Study Group. Efficacy and safety of recombinant human activated protein C for severe sepsis. N Engl J Med 2001; 344: 699709.
  • 129
    Aoki N, Matsuda T, Saito H, Takatsuki K, Okajima K, Takahashi H, Takamatsu J, Asakura H, Ogawa N. A comparative double-blind randomized trial of activated protein C and unfractionated heparin in the treatment of disseminated intravascular coagulation. Int J Hematol 2002; 75: 5407.
  • 130
    Saito H, Maruyama I, Shimazaki S, Yamamoto Y, Aikawa N, Ohno R, Hirayama A, Matsuda T, Asakura H, Nakashima M, Aoki N. Efficacy and safety of recombinant human soluble thrombomodulin (ART-123) in disseminated intravascular coagulation: results of a phase III, randomized, double-blind clinical trial. J Thromb Haemost 2007; 5: 3141.
  • 131
    Abraham E, Reinhart K, Opal S, Demeyer I, Doig C, Rodriguez AL, Beale R, Svoboda P, Laterre PF, Simon S, Light B, Spapen H, Stone J, Seibert A, Peckelsen C, De Deyne C, Postier R, Pettillä V, Artioas A, Percell SR, et al. ; OPTIMIST Trial Study Group. Efficacy and safety of tifacogin (recombinant tissue factor pathway inhibitor) in severe sepsis: a rendomaized controlled trial. JAMA 2003; 290: 23847.
  • 132
    Nemerson Y, Turitto VT. The effect of flow on hemostasis and thrombosis. Thromb Haemost 1991; 66: 2726.
  • 133
    Falati S, Gross P, Merrill-Skoloff G, Furie BC, Furie B. Real-time in vivo imaging of platelets, tissue factor and fibrin during arterial thrombus formation in the mouse. Nat Med 2002; 8: 117581.
  • 134
    Nishimura S, Manabe I, Nagasaki M, Seo K, Yamashita H, Hosoya Y, Ohsugi M, Tobe K, Kadowaki T, Nagai R, Sugiura S. In vivo imaging in mice reveals local cell dynamics and inflammation in obese adipose tissue. J Clin Invest 2008; 118: 71021.
  • 135
    Kay MA, Landen CN, Rothenberg SR, Taylor A, Leland F, Wiehle S, Fang B, Bellinger D, Finegold M, Thompson AR. In vivo hepatic gene therapy: complete albeit transient correction of factor IX deficiency in hemophilia B dogs. Proc Natl Acad Sci USA 1994; 91: 23537.
  • 136
    Chuah MK, Schiedner G, Thorrez L, Brown B, Johnston M, Gillijns V, Hertel S, von Rooijen N, Lillicrap D, Collen D, VandenDriessche T, Kochanek S. Therapeutic factor VIII levels and negligible toxicity in mouse and dog models of hemophilia A following gene therapy with high-capacity adenoviral vectors. Blood 2003; 101: 173443.
  • 137
    Manno CS, Pierce GF, Arruda VR, Glader B, Ragni M, Rasko JJ, Ozelo MC, Hoots K, Blatt P, Konkle B, Dake M, Kaye R, Razavi M, Zajko A, Zehnder J, Rustagi PK, Nakai H, Chew A, Leonard D, Wright JF, et al. Successful transduction of liver in hemophilia by AAV-Factor IX and limitations imposed by the host immune response. Nat Med 2006; 12: 3427.
  • 138
    Gailani D, Renné T. Intrinsic pathway of coagulation and arterial thrombosis. Arterioscler Thromb Vasc Biol 2007; 27: 250713.