Summary: Agents that cause clotting.

Top Publications

  1. Acharya S, Dimichele D. Rare inherited disorders of fibrinogen. Haemophilia. 2008;14:1151-8 pubmed publisher
    ..Rare Bleeding Disorder Registries developed through the United States and international efforts hopefully will encourage development and licensure of safer, effective products. ..
  2. Mancuso M, Graca L, Auerswald G, Santagostino E. Haemophilia care in children--benefits of early prophylaxis for inhibitor prevention. Haemophilia. 2009;15 Suppl 1:8-14 pubmed publisher
    ..Practical approaches to early prophylaxis as implemented in the haemophilia centres in Milan and Bremen are discussed in this respect...
  3. Goral V, Hsieh Y, Petzold O, Clark J, Yuen P, Faris R. Perfusion-based microfluidic device for three-dimensional dynamic primary human hepatocyte cell culture in the absence of biological or synthetic matrices or coagulants. Lab Chip. 2010;10:3380-6 pubmed publisher
    ..polarity and hepatocyte transport function in vitro without the addition of biological or synthetic matrices or coagulants. A unique feature of our dynamic cell culture device is the creation of a microenvironment, without the addition ..
  4. Dumont J, Liu T, Low S, Zhang X, Kamphaus G, Sakorafas P, et al. Prolonged activity of a recombinant factor VIII-Fc fusion protein in hemophilia A mice and dogs. Blood. 2012;119:3024-30 pubmed publisher
    ..5- to 2-fold longer than rFVIII. rFVIIIFc was well tolerated in both species. Thus, the rescue of FVIII by Fc fusion to provide prolonged protection presents a novel pathway for FVIII catabolism, and warrants further investigation. ..
  5. Collins P, Bjorkman S, Fischer K, Blanchette V, Oh M, Schroth P, et al. Factor VIII requirement to maintain a target plasma level in the prophylactic treatment of severe hemophilia A: influences of variance in pharmacokinetics and treatment regimens. J Thromb Haemost. 2010;8:269-75 pubmed publisher
  6. Björkman S, Folkesson A, Jönsson S. Pharmacokinetics and dose requirements of factor VIII over the age range 3-74 years: a population analysis based on 50 patients with long-term prophylactic treatment for haemophilia A. Eur J Clin Pharmacol. 2009;65:989-98 pubmed publisher
    ..However, plausible starting doses for most patients would be 1,000 U every other day. FVIII levels should then be checked for dose adjustment. ..
  7. Warren O, Simon B. Massive, fatal, intracardiac thrombosis associated with prothrombin complex concentrate. Ann Emerg Med. 2009;53:758-61 pubmed publisher
    ..Thrombogenic complications of treatment with prothrombin complex concentrate have been reported before. Caution should be used when using prothrombin complex concentrates for reversal of anticoagulation. ..
  8. Pipe S. Recombinant clotting factors. Thromb Haemost. 2008;99:840-50 pubmed publisher
    ..In turn, the ongoing development of new recombinant clotting factor concentrates is providing alternatives for patients with other inherited bleeding disorders. ..
  9. Sadler J. Biochemistry and genetics of von Willebrand factor. Annu Rev Biochem. 1998;67:395-424 pubmed

More Information


  1. Ganter M, Hofer C. Coagulation monitoring: current techniques and clinical use of viscoelastic point-of-care coagulation devices. Anesth Analg. 2008;106:1366-75 pubmed publisher
    ..To ensure optimal accuracy and performance, standardized procedures for blood sampling and handling, strict quality controls and trained personnel are required. ..
  2. Risebrough N, Oh P, Blanchette V, Curtin J, Hitzler J, Feldman B. Cost-utility analysis of Canadian tailored prophylaxis, primary prophylaxis and on-demand therapy in young children with severe haemophilia A. Haemophilia. 2008;14:743-52 pubmed publisher
    ..In conclusion, prophylaxis will substantially improve clinical outcomes and quality of life compared to Demand treatment, but with substantial cost...
  3. Keeling D, Tait C, Makris M. Guideline on the selection and use of therapeutic products to treat haemophilia and other hereditary bleeding disorders. A United Kingdom Haemophilia Center Doctors' Organisation (UKHCDO) guideline approved by the British Committee for Standards in H. Haemophilia. 2008;14:671-84 pubmed publisher
    ..They include details of therapeutic products available in the UK and they update and replace previous United Kingdom Haemophilia Centre Doctors' Organisation guidelines. ..
  4. den Uijl I, Fischer K. Including the life-time cumulative number of joint bleeds in the definition of primary prophylaxis. Thromb Haemost. 2008;99:965; author reply 966-8 pubmed publisher
  5. Schobess R, Kurnik K, Friedrichs F, Halimeh S, Krümpel A, Bidlingmaier C, et al. Effects of primary and secondary prophylaxis on the clinical expression of joint damage in children with severe haemophilia A. Results of a multicenter non-concurrent cohort study. Thromb Haemost. 2008;99:71-6 pubmed publisher
    ..77). No conclusion can be drawn from the data presented herein whether primary prophylaxis or an early start of secondary prophylaxis is superior with respect to joint outcome in children with severe HA...
  6. Munnix I, Harmsma M, Giddings J, Collins P, Feijge M, Comfurius P, et al. Store-mediated calcium entry in the regulation of phosphatidylserine exposure in blood cells from Scott patients. Thromb Haemost. 2003;89:687-95 pubmed
    ..patient V.W. is a new case of Scott syndrome, ii. Ca(2+) entry in the platelets and lymphoblasts from both Scott patients is normal, and iii. elevated [Ca(2+)](i) as caused by SMCE is not sufficient to trigger PS exposure. ..
  7. Shapiro A, Korth Bradley J, Poon M. Use of pharmacokinetics in the coagulation factor treatment of patients with haemophilia. Haemophilia. 2005;11:571-82 pubmed
    ..We highlight the importance of considering an individual pharmacokinetic study in all patients starting a new coagulation product. ..
  8. Hoots W, Nugent D. Evidence for the benefits of prophylaxis in the management of hemophilia A. Thromb Haemost. 2006;96:433-40 pubmed
  9. Haas T, Fries D, Velik Salchner C, Reif C, Klingler A, Innerhofer P. The in vitro effects of fibrinogen concentrate, factor XIII and fresh frozen plasma on impaired clot formation after 60% dilution. Anesth Analg. 2008;106:1360-5, table of contents pubmed publisher
    ..Supplementation of fibrinogen restored all ROTEM parameters after dilution. This effect was partially enhanced by adding FXIII and was significantly stronger than for FFP substitution. ..
  10. Pollmann H, Externest D, Ganser A, Eifrig B, Kreuz W, Lenk H, et al. Efficacy, safety and tolerability of recombinant factor VIII (REFACTO) in patients with haemophilia A: interim data from a postmarketing surveillance study in Germany and Austria. Haemophilia. 2007;13:131-43 pubmed
    ..These results emphasize the benefit of postmarketing surveillance and, overall, this study confirms the efficacy, safety and tolerability of REFACTO in the treatment of patients with haemophilia A. ..
  11. Almegren M. Reversal of direct oral anticoagulants. Vasc Health Risk Manag. 2017;13:287-292 pubmed publisher
    ..This review summarizes the current evidence for nonspecific and specific reversal of DOACs. ..
  12. Mariani G, Dolce A, Batorova A, Auerswald G, Schved J, Siragusa S, et al. Recombinant, activated factor VII for surgery in factor VII deficiency: a prospective evaluation - the surgical STER. Br J Haematol. 2011;152:340-6 pubmed publisher
    ..This indication is important especially in the case of major surgery. ..
  13. Raquet E, Stockschlader M, Dickneite G. Repeated infusions of VWF/FVIII concentrate: impact of VWF:FVIII ratio on FVIII trough and peak levels in a rabbit model. Haemophilia. 2011;17:808-14 pubmed publisher
    ..Half-life did not differ between the two concentrates. Animal model data suggest that exposure to elevated FVIII levels can be reduced through use of VWF/FVIII concentrates with higher VWF:FVIII ratios. ..
  14. Bilecen S, Peelen L, Kalkman C, Spanjersberg A, Moons K, Nierich A. Fibrinogen concentrate therapy in complex cardiac surgery. J Cardiothorac Vasc Anesth. 2013;27:12-7 pubmed publisher
    ..This study reports the initial clinical use of fibrinogen concentrate in complex cardiac surgery. A randomized clinical trial has been initiated to investigate the hemostatic role of fibrinogen concentrate in cardiac surgery. ..
  15. Westra D, Dorresteijn E, Beishuizen A, van den Heuvel L, Brons P, van de Kar N. The challenge of managing hemophilia A and STEC-induced hemolytic uremic syndrome. Pediatr Nephrol. 2013;28:349-52 pubmed publisher
    ..The role of factor VIII administration in the severe outcome of this disease is discussed. ..
  16. Javedani P, Horowitz B, Clark W, Lutsep H. Dabigatran etexilate: management in acute ischemic stroke. Am J Crit Care. 2013;22:169-76 pubmed publisher
    ..In this case report, we present a man who had an acute ischemic stroke while taking dabigatran and discuss the medical decision making regarding management issues. ..
  17. Ljung R, Karim F, Saxena K, Suzuki T, Arkhammar P, Rosholm A, et al. 40K glycoPEGylated, recombinant FVIIa: 3-month, double-blind, randomized trial of safety, pharmacokinetics and preliminary efficacy in hemophilia patients with inhibitors. J Thromb Haemost. 2013;11:1260-8 pubmed publisher
    ..The trial was registered at ( NCT00951405). ..
  18. Ramos M, Araujo E, Jucá T, Monteiro Moreira A, Vasconcelos I, Moreira R, et al. New insights into the complex mixture of latex cysteine peptidases in Calotropis procera. Int J Biol Macromol. 2013;58:211-9 pubmed publisher
    ..The purified enzymes exhibited plasma-clotting activity mediated by a thrombin-like mechanism. The set of results suggest the three isolated polypeptides correspond to different post-translationally processed forms of the same protein. ..
  19. Berntorp E. Pharmacokinetics of plasma-derived and recombinant factor IX: using population pharmacokinetics with sparse sampling data needs further study. Haemophilia. 2013;19:805-7 pubmed publisher
    ..Sven Björkman opened a new era in hemophilia prophylaxis. ..
  20. Hewson I, Makhmaloaf P. Management of third molar removal with a single dose of recombinant Factor IX (BeneFIX) and local measures in severe haemophilia B. Aust Dent J. 2010;55:322-4 pubmed publisher
    ..Oral surgery may be performed on patients with inherited bleeding disorders using minimal factors and local haemostatic measures. A study of this patient population has commenced at The Alfred Hospital. ..
  21. McQuilten Z, Bailey M, Cameron P, Stanworth S, Venardos K, Wood E, et al. Fibrinogen concentration and use of fibrinogen supplementation with cryoprecipitate in patients with critical bleeding receiving massive transfusion: a bi-national cohort study. Br J Haematol. 2017;179:131-141 pubmed publisher
    ..The findings indicate areas for practice improvement including timely administration of cryoprecipitate, which is the most common source of concentrated fibrinogen in Australia and New Zealand. ..
  22. Cuccuini W, Poitevin S, Poitevin G, Dignat George F, Cornillet Lefebvre P, Sabatier F, et al. Tissue factor up-regulation in proinflammatory conditions confers thrombin generation capacity to endothelial colony-forming cells without influencing non-coagulant properties in vitro. J Thromb Haemost. 2010;8:2042-52 pubmed publisher
    ..Our results suggest that EPC-based cell therapy may be associated with prothrombotic risk which could be limited by inhibiting TF without affecting the proangiogenic capacity of the cells. ..
  23. McAdow M, Kim H, Dedent A, Hendrickx A, Schneewind O, Missiakas D. Preventing Staphylococcus aureus sepsis through the inhibition of its agglutination in blood. PLoS Pathog. 2011;7:e1002307 pubmed publisher
    ..Together these results suggest that the combined administration of direct thrombin inhibitors and ClfA-antibodies that block S. aureus agglutination with fibrin may be useful for the prevention of staphylococcal sepsis in humans. ..
  24. Beckett A, Tien H. What's new in operative trauma surgery in the last 10 years. Curr Opin Crit Care. 2013;19:599-604 pubmed publisher
    ..The articles we review here are essential for the contemporary care of the severely injured trauma patient, whether they are card for in a level 1 trauma center or in a field hospital at the edge of a battlefield. ..
  25. Kosloski M, Pisal D, Mager D, Balu Iyer S. Allometry of factor VIII and informed scaling of next-generation therapeutic proteins. J Pharm Sci. 2013;102:2380-94 pubmed publisher
    ..quot; This approach might be generally applicable for predicting human PK of next-generational therapeutics. ..
  26. Giordano P, Franchini M, Lassandro G, Faienza M, Valente R, Molinari A. Issues in pediatric haemophilia care. Ital J Pediatr. 2013;39:24 pubmed publisher
  27. Jiang D, Cao Y, Xin H, Li X, Luo Z, Li Y. Asymmetric dimethylarginine induces tissue factor expression in monocytes via NF-kappaB-dependent pathway: Role in acute coronary syndromes. Atherosclerosis. 2009;205:554-60 pubmed publisher
    ..Our results suggest that elevated plasma level of ADMA induces TF expression in monocytes via NF-kappaB-dependent pathway, which contributes to procoagulant phenotype of circulating monocytes in ACS. ..
  28. Gollop N, Chilcott J, Benton A, Rayment R, Jones J, Collins P. National audit of the use of fibrinogen concentrate to correct hypofibrinogenaemia. Transfus Med. 2012;22:350-5 pubmed publisher
    ..001). Three venous and one arterial non-fatal thrombotic events were recorded in the patients treated with fibrinogen. Fibrinogen concentrate can be used to correct hypofibrinogenaemia and may reduce blood product usage. ..
  29. Michalska Krzanowska G. [Recombinant factor VIIa for treatment of postpartum hemorrhage due to uterine atony]. Ginekol Pol. 2010;81:544-8 pubmed
    ..In all presented cases, bleeding symptoms abated and hemodynamic stabilization was obtained shortly after the drug administration. NovoSeven is a very effective agent in the treatment of postpartum hemorrhage. ..
  30. Sridharan M, Wysokinski W, Pruthi R, Oyen L, Freeman W, Rabinstein A, et al. Periprocedural warfarin reversal with prothrombin complex concentrate. Thromb Res. 2016;139:160-5 pubmed publisher
  31. Schöchl H, Grassetto A, Schlimp C. Management of hemorrhage in trauma. J Cardiothorac Vasc Anesth. 2013;27:S35-43 pubmed publisher
    ..However, there is increased awareness that coagulation factor concentrate could be beneficial in the treatment of trauma-induced coagulopathy. ..
  32. Holster I, Hunfeld N, Kuipers E, Kruip M, Tjwa E. On the treatment of new oral anticoagulant-associated gastrointestinal hemorrhage. J Gastrointestin Liver Dis. 2013;22:229-31 pubmed
    ..The generalizability of these recommendations needs to be confirmed in future studies. ..
  33. Spahn D. Severe bleeding in surgical and trauma patients: the role of fibrinogen replacement therapy. Thromb Res. 2012;130 Suppl 2:S15-9 pubmed publisher
  34. Wang J, Shi X, Yang J, Hu D. In vitro anticoagulation monitoring of low-molecular-weight heparin. Chin Med J (Engl). 2009;122:1199-202 pubmed
    ..Four coagulants, kaolin, diatomite, glass bead, and magnetic stick, were used to determine the activated clotting time of the ..
  35. Makris M, Hay C, Gringeri A, d Oiron R. How I treat inhibitors in haemophilia. Haemophilia. 2012;18 Suppl 4:48-53 pubmed publisher
    ..Acute bleeding is treated using one of two bypassing agents, which exhibit similar efficacy and safety. Surgery in inhibitor patients is challenging and should only be carried out in experienced centres. ..
  36. Throndson R, Baker D, Kennedy P, McDaniel K. Pseudotumor of hemophilia in the mandible of a patient with hemophilia A. Oral Surg Oral Med Oral Pathol Oral Radiol. 2012;113:229-33 pubmed publisher
    ..Fewer than 20 cases have been reported in the maxillofacial region. We report a rare case occurring in the mandible of a 14-year-old boy who presented with considerable expansion and displacement of teeth. ..
  37. Shakhnovich V, Daniel J, Wicklund B, Kearns G, Neville K. Use of pharmacokinetic modelling to individualize FFP dosing in factor V deficiency. Haemophilia. 2013;19:251-5 pubmed publisher
  38. Napenas J, Hong C, Brennan M, Furney S, Fox P, Lockhart P. The frequency of bleeding complications after invasive dental treatment in patients receiving single and dual antiplatelet therapy. J Am Dent Assoc. 2009;140:690-5 pubmed
    ..The risks of altering or discontinuing use of antiplatelet medications far outweigh the low risk of postoperative oral bleeding complications resulting from dental procedures. ..
  39. Tessitore E, Schatlo B, Schaller C, Schonauer C. Fibrillary structure is key for hemostasis: a similar effect of collagen fleece and oxidized cellulose on experimental hemorrhagic brain injury. J Neurol Surg A Cent Eur Neurosurg. 2012;73:89-92 pubmed publisher
    ..ORC and CF significantly reduce blood loss from hemorrhagic contusions. Our data suggest that they effectively reduce bleeding time. We advocate the use of hemostatic material for limiting bleeding from superficial cortical lesions. ..
  40. Song J, Lim K, Kang S, Noh J, Kim K, Bae O, et al. Procoagulant and prothrombotic effects of the herbal medicine, Dipsacus asper and its active ingredient, dipsacus saponin C, on human platelets. J Thromb Haemost. 2012;10:895-906 pubmed publisher
    ..These results demonstrate that herbal medicines or natural products such as DA or DSC might have prothrombotic risks through procoagulant activation of platelets. ..
  41. Song H, von Heymann C, Jespersen C, Karkouti K, Korte W, Levy J, et al. Safe application of a restrictive transfusion protocol in moderate-risk patients undergoing cardiac operations. Ann Thorac Surg. 2014;97:1630-5 pubmed publisher
    ..This strategy has significant potential to reduce transfusion and resource utilization in these patients, standardize transfusion practices across institutions, and increase the safety of cardiac operations. ..
  42. Quader M, Rusina Z, Lewis N, Martin L, Katlaps G. Left ventricular assist device and heart transplantation in hemophilia a patient. Ann Thorac Surg. 2013;96:2245-7 pubmed publisher
    ..Preparation for surgery with factor VIII supplementation, intraoperative conduct of surgery, and challenges of postoperative course are described with a brief literature review. ..
  43. van Vliet H, Kappers Klunne M, Leebeek F, Michiels J. PFA-100 monitoring of von Willebrand factor (VWF) responses to desmopressin (DDAVP) and factor VIII/VWF concentrate substitution in von Willebrand disease type 1 and 2. Thromb Haemost. 2008;100:462-8 pubmed
  44. Ferraz N, Hong J, Karlsson Ott M. Procoagulant behavior and platelet microparticle generation on nanoporous alumina. J Biomater Appl. 2010;24:675-92 pubmed publisher
  45. Harenberg J, Marx S, Krämer R, Giese C, Weiss C. Determination of an international sensitivity index of thromboplastin reagents using a WHO thromboplastin as calibrator for plasma spiked with rivaroxaban. Blood Coagul Fibrinolysis. 2011;22:637-41 pubmed publisher
    ..In conclusion, the determination of an ISI for rivaroxaban using a WHO thromboplastin reagent is required for commercial thromboplastin reagents. The manual Kolle-Hook method and an ordinary linear regression analysis should be adopted...
  46. Zanon E, Milan M, Gamba G, Ambaglio C, Saggiorato G, Spiezia L, et al. Activated prothrombin complex concentrate (FEIBA®) for the treatment and prevention of bleeding in patients with acquired haemophilia: A sequential study. Thromb Res. 2015;136:1299-302 pubmed publisher
    ..Prolonging the treatment with lower doses of aPCC beyond the initial phase in patients with AHA in whom the titre of FVIII inhibitor is still high is likely to safely prevent further bleeding complications. ..
  47. Osterud B. Tissue factor/TFPI and blood cells. Thromb Res. 2012;129:274-8 pubmed publisher
    ..In this short review the distribution of TF and TFPI in blood is discussed. ..
  48. Brown D, Kouides P. Diagnosis and treatment of inherited factor X deficiency. Haemophilia. 2008;14:1176-82 pubmed publisher
    ..There is no specific FX replacement product yet readily available, but fresh frozen plasma and prothrombin complex concentrates can be used for treatment of bleeding symptoms and preparation for surgery. ..
  49. de Wee E, Leebeek F, Eikenboom J. Diagnosis and management of von Willebrand disease in The Netherlands. Semin Thromb Hemost. 2011;37:480-7 pubmed publisher
    ..Recently a study on moderate and severe VWD (the Willebrand in Netherlands study) was initiated to obtain more insight on VWD diagnosis, treatment, and the burden of the disease. ..
  50. Narins C, Zareba W, Rocco V, McNitt S. A prospective, randomized trial of topical hemostasis patch use following percutaneous coronary and peripheral intervention. J Invasive Cardiol. 2008;20:579-84 pubmed
    ..In conclusion, this trial supports the concept that the polysaccharide-based SafeSeal Patch enhances access site hemostasis following endovascular intervention. ..
  51. Kao C, Lin C, Yu I, Tao M, Wu H, Shi G, et al. FIX-Triple, a gain-of-function factor IX variant, improves haemostasis in mouse models without increased risk of thrombosis. Thromb Haemost. 2010;104:355-65 pubmed publisher
    ..These studies suggest that FIX-Triple is not thrombogenic at therapeutic levels and is a potential therapeutic substitute for FIX-WT. ..
  52. Bjorkman S. Pharmacokinetics of plasma-derived and recombinant factor IX - implications for prophylaxis and on-demand therapy. Haemophilia. 2013;19:808-13 pubmed publisher
    ..However, the putative relationship between FIX:C trough level and therapeutic outcome has never been confirmed in a clinical trial. Comparative studies on prophylaxis with different types of FIX are needed. ..
  53. Moghimi B, Sack B, Nayak S, Markusic D, Mah C, Herzog R. Induction of tolerance to factor VIII by transient co-administration with rapamycin. J Thromb Haemost. 2011;9:1524-33 pubmed publisher
    ..Immune suppression with rapamycin was mild and highly transient, as the mice regained immune competence within a few weeks. ..