eMedicine Specialties > Hematology > Coagulation, Hemostasis, and Disorders
Factor IX: Multimedia
Updated: Aug 30, 2007
Multimedia
 | Media file 1: Obituary in the Salem Gazette (Massachusetts) of a 19-year-old man, March 22, 1796. |
 | Media file 2: Major components of the factor IX structure. |
 | Media file 3: Vitamin K–dependent carboxylation of precursor factor IX to procoagulant factor IX. Carboxylation of glutamate (Glu) to gamma-carboxyglutamate (Gla) residues in the precursor protein of the vitamin K–dependent factors occurs in the endoplasmic reticulum of the hepatocyte. Reduced vitamin K is oxidized in this process. Warfarin prevents the reduction and recycling of oxidized vitamin K. |
 | Media file 4: The hemostatic pathway: role of factor IX. |
 | Media file 5: Activation of factor IX and function of the intrinsic tenase complex. Activation of factor IX is followed by formation of the intrinsic tenase complex, which activates factor X to activated factor X, leading to a second and larger burst of thrombin production during activation of hemostasis. |
 | Media file 6: Cell surfaced-directed hemostasis. Initially, a small amount of thrombin is generated on the surface of the tissue factor–bearing (TF-bearing) cell. Following amplification, the second burst generates a larger amount of thrombin, leading to fibrin (clot) formation. (Adapted from Hoffman and Monroe, Thromb Haemost 2001, 85(6): 958-65.) |
 | Media file 7: Possible genetic outcomes in individuals carrying the hemophilic gene. |
 | Media file 8: Teenage boy with bleeding into right thigh, both knees, and ankles. |
 | Media file 9: Older adult man with chronic fused extended knee following open drainage of right knee bleed many years previously. |
 | Media file 10: Severe bilateral hemophilic arthropathy and muscle wasting. Three puncture sites demonstrate attempts to aspirate a recent bleed into the knee joint. |
 | Media file 11: Chronic severe arthritis, fusion, and loss of cartilage and joint space with deformities in the knees. Findings are of advanced hemophilic arthropathy. |
 | Media file 12: Chronic severe arthritis, fusion, and loss of cartilage and joint space with deformities in the elbow. Findings are of advanced hemophilic arthropathy. |
 | Media file 13: Hemophilic knee at surgery with synovial proliferation caused by repeated bleeding and requiring synovectomy. |
 | Media file 14: Large amount of vascular synovium removed during knee surgery (same patient depicted in Picture 7). |
 | Media file 15: Microscopic appearance of synovial proliferation and high vascularity. If stained with iron, would show diffuse deposits. Iron-laden macrophages are present. |
 | Media file 16: Male patient presenting with a slowly expanding abdominal and flank mass with increasing pain, inability to eat, weight loss, and weakness of the lower extremity. |
 | Media file 17: Plain radiograph of the pelvis showing a large lytic area. |
 | Media file 18: Intravenous pyelogram showing extreme displacement of the left kidney and ureter by the pseudocyst. |
 | Media file 19: Dissection of a pseudocyst. |
 | Media file 20: Transected pseudocyst with old chocolate brown–black blood. |
 | Media file 21: Large pseudocyst involving left proximal femur. |
 | Media file 22: Transected pseudocyst (following disarticulation of the lower left extremity because of vascular compromise, nerve damage, loss of bone, and nonfunctional lower left extremity) showing old black-brown blood, residual muscle, and bone. |
 | Media file 23: Extensive spontaneous abdominal wall hematoma and thigh hemorrhage in a previously healthy older man with an acquired factor VIII inhibitor. |
 | Media file 24: Extensive spontaneous abdominal wall hematoma and thigh hemorrhage in a previously healthy older man with an acquired factor VIII inhibitor. |
 | Media file 25: Application of Velcro tourniquet followed by self-infusion of concentrate as part of home therapy. |
 | Media file 26: Application of Velcro tourniquet followed by self-infusion of concentrate as part of home therapy. |
 | Media file 27: Quality of life at summer camp. |
More on Factor IX |
| Overview: Factor IX |
| Differential Diagnoses & Workup: Factor IX |
| Treatment & Medication: Factor IX |
| Follow-up: Factor IX |
 Multimedia: Factor IX |
| References |
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References
Bauer KA, Kass BL, ten Cate H, et al. Factor IX is activated in vivo by the tissue factor mechanism. Blood. Aug 15 1990;76(4):731-6. [Medline].
Hoffman M, Monroe DM 3rd. A cell-based model of hemostasis. Thromb Haemost. Jun 2001;85(6):958-65. [Medline].
Roberts HR, Monroe DM III, Hoffman M. Molecular biology and biochemistry of the coagulation factors and pathways of hemostasis. In: Beutler E, Lichtman M, Coller B, et al, eds. Williams Hematology. 2001. 6th ed. New York, NY: McGraw-Hill Professional; 2001:1409-34.
Quadros L, Ghosh K, Shetty S. A common G10430A mutation (Gly 60 Ser) in the factor IX gene describes the presence of moderate and mild hemophilia B in the majority of the Gujarati population. Ann Hematol. May 2007;86(5):377-9. [Medline].
Kulkarni R, Lusher J. Perinatal management of newborns with haemophilia. Br J Haematol. Feb 2001;112(2):264-74. [Medline].
Kulkarni R, Lusher JM, Henry RC, Kallen DJ. Current practices regarding newborn intracranial haemorrhage and obstetrical care and mode of delivery of pregnant haemophilia carriers: a survey of obstetricians, neonatologists and haematologists in the United States, on behalf of the National Hemop. Haemophilia. Nov 1999;5(6):410-5. [Medline].
Christophe OD, Lenting PJ, Cherel G, et al. Functional mapping of anti-factor IX inhibitors developed in patients with severe hemophilia B. Blood. Sep 1 2001;98(5):1416-23. [Medline].
Poon MC. Clotting Factor IX in Hemophilia B: Global Experience. Paper presented at: XVIII Congress of the International Society on Thrombosis and Haemostasis. July 6-12, 2001;Paris, France.
Roth DA, Kessler CM, Pasi KJ, et al. Human recombinant factor IX: safety and efficacy studies in hemophilia B patients previously treated with plasma-derived factor IX concentrates. Blood. Dec 15 2001;98(13):3600-6. [Medline].
Azzi A, De Santis R, Morfini M, et al. TT virus contaminates first-generation recombinant factor VIII concentrates. Blood. Oct 15 2001;98(8):2571-3. [Medline].
Poon MC. Pharmacokinetics of factors IX, recombinant human activated factor VII and factor XIII. Haemophilia. Nov 2006;12 Suppl 4:61-9.
Hedner U, Ginsburg D, Lusher JM, High KA. Congenital Hemorrhagic Disorders: New Insights into the Pathophysiology and Treatment of Hemophilia. Hematology (Am Soc Hematol Educ Program). 2000;241-65.
Coukos G, Rubin SC. Gene therapy for ovarian cancer. Oncology (Huntingt). Sep 2001;15(9):1197-204, 1207; discussion 1207-8. [Medline].
Ponder KP. Gene therapy for hemophilia. Curr Opin Hematol. Sep 2006;13(5):301-7. [Medline].
Treisman GJ, Angelino AF, Hutton HE. Psychiatric issues in the management of patients with HIV infection. JAMA. Dec 12 2001;286(22):2857-64. [Medline].
Di Bisceglie AM. SEN and sensibility: interactions between newly discovered and other hepatitis viruses?. Lancet. Dec 8 2001;358(9297):1925-6. [Medline].
Rigas B, Hasan I, Rehman R, et al. Effect on treatment outcome of coinfection with SEN viruses in patients with hepatitis C. Lancet. Dec 8 2001;358(9297):1961-2. [Medline].
Lauer GM, Walker BD. Hepatitis C virus infection. N Engl J Med. Jul 5 2001;345(1):41-52. [Medline].
MediView. Recombinant therapy enhances safety and quality of life for hemophilia patients. Paper presented at: 53rd Annual Meeting of the National Hemophilia Foundation. November 16, 2001:Nashville, Tennessee.
Senior K. New variant CJD fears threaten blood supplies. Lancet. Jul 28 2001;358(9278):304. [Medline].
Feuerstein GZ, Nichols AJ, Church WR. Novel murine monoclonal antifactor IX/IXa (BC2) is a potent anticoagulant with "self limiting" inhibition of hemostasis. Circulation. 1997;96:142.
Kjalke M, Monroe DM, Hoffman M, et al. Active site-inactivated factors VIIa, Xa, and IXa inhibit individual steps in a cell-based model of tissue factor-initiated coagulation. Thromb Haemost. Oct 1998;80(4):578-84. [Medline].
Spanier TB, Oz MC, Minanov OP, et al. Heparinless cardiopulmonary bypass with active-site blocked factor IXa: a preliminary study on the dog. J Thorac Cardiovasc Surg. May 1998;115(5):1179-88. [Medline].
Bajzar L, Manuel R, Nesheim ME. Purification and characterization of TAFI, a thrombin-activable fibrinolysis inhibitor. J Biol Chem. Jun 16 1995;270(24):14477-84. [Medline].
Bajzar L, Nesheim ME, Tracy PB. The profibrinolytic effect of activated protein C in clots formed from plasma is TAFI-dependent. Blood. Sep 15 1996;88(6):2093-100. [Medline].
Bouma BN, von dem Borne PA, Meijers JC. Factor XI and protection of the fibrin clot against lysis--a role for the intrinsic pathway of coagulation in fibrinolysis. Thromb Haemost. Jul 1998;80(1):24-7. [Medline].
Brooks M. A review of canine inherited bleeding disorders: biochemical and molecular strategies for disease characterization and carrier detection. J Hered. Jan-Feb 1999;90(1):112-8. [Medline].
Broze GJ Jr, Higuchi DA. Coagulation-dependent inhibition of fibrinolysis: role of carboxypeptidase-U and the premature lysis of clots from hemophilic plasma. Blood. Nov 15 1996;88(10):3815-23. [Medline].
Economides DL, Kadir RA, Lee CA. Inherited bleeding disorders in obstetrics and gynaecology. Br J Obstet Gynaecol. Jan 1999;106(1):5-13. [Medline].
The fibrinolytic system and thrombolytic agents. In: Bachmann F, ed. Fibrinolytics and Antifibrinolytics. New York, NY: Springer-Verlag; 2001:3-15.
Green D. Complications associated with the treatment of haemophiliacs with inhibitors. Haemophilia. Sep 1999;5 Suppl 3:11-7. [Medline].
Hoots K, Canty D. Clotting factor concentrates and immune function in haemophilic patients. Haemophilia. Sep 1998;4(5):704-13. [Medline].
Kasper CK, Costa E, Silva M. Registry of Clotting Factor Concentrates. World Federation of Hemophilia;1998:1-8.
Key NS, Aledort LM, Beardsley D, et al. Home treatment of mild to moderate bleeding episodes using recombinant factor VIIa (Novoseven) in haemophiliacs with inhibitors. Thromb Haemost. Dec 1998;80(6):912-8. [Medline].
Knobe KE, Persson KE, Sjörin E, Villoutreix BO, Ljung RC. Functional analysis of the factor IX epidermal growth factor-like domain mutation Ile66Thr associated with mild hemophilia B. Pathophysiol Haemost Thromb. 2006;35(5):370-5. [Medline].
Lillicrap D. Molecular diagnosis of inherited bleeding disorders and thrombophilia. Semin Hematol. Oct 1999;36(4):340-51. [Medline].
Mosnier LO, von dem Borne PA, Meijers JC, et al. Plasma TAFI levels influence the clot lysis time in healthy individuals in the presence of an intact intrinsic pathway of coagulation. Thromb Haemost. Nov 1998;80(5):829-35. [Medline].
Qian J, Collins M, Sharpe AH, Hoyer LW. Prevention and treatment of factor VIII inhibitors in murine hemophilia A. Blood. Feb 15 2000;95(4):1324-9. [Medline].
Racoosin JA, Kessler CM. Bleeding episodes in HIV-positive patients taking HIV protease inhibitors: a case series. Haemophilia. Jul 1999;5(4):266-9. [Medline].
Redlitz A, Tan AK, Eaton DL, Plow EF. Plasma carboxypeptidases as regulators of the plasminogen system. J Clin Invest. Nov 1995;96(5):2534-8. [Medline].
Shapiro AD, Gilchrist GS, Hoots WK, et al. Prospective, randomised trial of two doses of rFVIIa (NovoSeven) in haemophilia patients with inhibitors undergoing surgery. Thromb Haemost. Nov 1998;80(5):773-8. [Medline].
Srivastava A, Chandy M, Sunderaj GD, et al. Low-dose intermittent factor replacement for post-operative haemostasis in haemophilia. Haemophilia. Nov 1998;4(6):799-801. [Medline].
Tedgard U, Ljung R, McNeil TF. Reproductive choices of haemophilia carriers. Br J Haematol. Aug 1999;106(2):421-6. [Medline].
Teitel JM. Recombinant factor VIIa versus aPCCs in haemophiliacs with inhibitors: treatment and cost considerations. Haemophilia. Sep 1999;5 Suppl 3:43-9. [Medline].
Thomson AR. Molecular biology of F IX. In: Colman RW, George JN, Hirsh J, et al, eds. Hemostasis and Thrombosis: Basic Principles and Clinical Practice. 4th ed. Philadelphia, Pa: Lippincott-Raven Pub; 2001:123-34.
Tock B, Drohan W, Hess J, et al. Haemophilia and advanced fibrin sealant technologies. Haemophilia. Jul 1998;4(4):449-55. [Medline].
Warrier I, Ewenstein BM, Koerper MA, et al. Factor IX inhibitors and anaphylaxis in hemophilia B. J Pediatr Hematol Oncol. Jan-Feb 1997;19(1):23-7. [Medline].
Wildgoose P, Nemerson Y, Hansen LL, et al. Measurement of basal levels of factor VIIa in hemophilia A and B patients. Blood. Jul 1 1992;80(1):25-8. [Medline].
Further Reading
Keywords
hemophilia B, Christmas disease, hemophiliac, hemophilia, blood factors, factor 9, FIX, bleeding disorder, blood disease, blood disorder, hemarthrosis, hematomas, mucocutaneous bleeding, inherited blood disease, familial bleeding disorder, familial blood disease, factor replacement therapy
