COAGULATION FACTOR REPLACEMENT Deficient factor t1/2 in vivo I 3-4 d Factor level adequate for hemostasis 100 mg/dL 1, 1, p p 1033 Product name or abbreviationa Loading dose Maintenance dose Cryoprecipitate 1 bag/7 kg 4, p 1 bag/15 kg qd 8, 756; k p 1108; k FFP, cryopoor plasma 3, p 24 10-20 mL/kg 4, 3 mL/kg q 24 h 1, p 756; 1, p 841 p 841 Factor IX Complex (Profilnine SD [Alpha Therapeutic Corp.]) FFP, cryopoor plasma 3, p 24 20 IU/kg 6, m 5 IU/kg q 24 h 6, m 20 mL/kg 1, p 3-6 mL/kg q 12 h 843 1, p 843 FFP, cryopoor plasma 3, p 24 10-20 mL/kg 4, 20 mL/kg q 4 h or 40 mL/kg q 8 h 14, 1033 II 2-5 d 20-40% 1, p 1, p 1033 1033 V 1536 h 25-30% 1, p 843 1, pp 843, 1033 VII 4-7 h 10-20% 1; pp 1, p 845, 1033 p 756; j 1033 VIII 9-18 h 1; pp b 100%d 819, 1033 Factor VIIa (Recombinant) (NovoSeven [Novo Nordisk]) Antihemophilic Factor (Human) (MONARC-M [American Red Cross]), Antihemophilic Factor (Recombinant) (Helixate FS [Aventis Behring], Recombinate [Baxter]) Cryoprecipitate 15-30 mcg/kg 16 50 IU/kgc 50 IU/kg q 12 h, or 50 IU/kg/12 h by continuous infusionc 0.5 bag/kg 3, pp 0.5 bag/kg q 12 he 50 IU FVIII:C/kg q 12 h, or 50 IU FVIII:C/kg/12 h by continuous infusionc 26-27 IX 2024 h 1; pp 819, 1033 100%d Antihemophilic Factor/von Willebrand Factor Complex (Human) (Humate-P [Aventis Behring GmbH]) Factor IX Complex, Factor IX (Recombinant) (BeneFIX [Wyeth]), Factor IX (Human) (AlphaNine SD [Alpha Therapeutic Corp.]) 15-30 µg/kg q 46 h 16 50 IU FVIII:C/kgc 100 IU/kgc 50 IU/kg q 12 hc Deficient factor t1/2 in vivo X 2042 h Factor level adequate for hemostasis 10-40% 1, p Product name or abbreviationa Loading dose Maintenance dose Factor IX Complex (Profilnine SD [Alpha Therapeutic Corp.]) FFP, cryopoor plasma 3, p 24 FFP, cryopoor plasma 3, p 24 10-20 IU/kg 4, 8-10 IU/kg q 24 h p 756; n 10, n 10-20 mL/kg 8, 3-6 mL/kg q 12 h p 212 8, p 212 Not applicable Not applicable Not applicable Not applicable 1-5%g Cryoprecipitate 1, p 1 unit/10-20 kgf 1 unit/10-20 kg q 3-4 wk 12 FFP 500 mL 8, p 1107 Antihemophilic Factor/von Willebrand Factor Complex (Human) (Humate-P [Aventis Behring GmbH]) Antihemophilic Factor (Human) (Alphanate [Alpha Therapeutic]) Cryoprecipitate 60-80 IU VWF:RCo/kg 2-3 mL/kg q 4-6 wk 12, h 40-60 IU VWF:RCo/kg q 8-12 h 1, p 833 848 1, p 848 XI 4080 h 15-25% 1, p 1033 10-20 mL/kg 4, p 756 5 mL/kg q 12-24 h 12 1, p 1033 XII 4852 h 1, p 1033 XIII 12 d 1, p 238 1033 Von Willebrand Factor (VWF) 3-5 h 25-50% 1, p 5, p 27 1033 1, p 833 60-70 IU Factor VIII:C/kgi 40-50 IU Factor VIII:C/kg q 12 h 1, 1-2 bags/10 kg 1, p 832 0.7-1.4 bags/10 kg q 8-12 hi p 832 Notes a Product names are from references 17 and 18. Abbreviation Cryopoor plasma Cryoprecipitate FFP Proper name 17, 18 Plasma, Cryoprecipitate Reduced Cryoprecipitated AHF Fresh Frozen Plasma Pooled Plasma, Solvent/Detergent Treated, may be substituted for Fresh Frozen Plasma (FFP). b Other recommendations: 5 mL/kg q 6-24 h 12, 5 mL/kg q 6 h 15, 10-15 mL/kg q 3-4 d 11. c For the most serious bleeding. See reference 1, p 819, for details. d 100% for CNS, traumatic, surgical, or retroperitoneal bleeding; less for less severe bleeding. See reference 1, p 819, for details. e The maintenance dose is the same as the loading dose 1, p 819. f The loading dose of Factor XIII concentrate recommended by Giangrande was about the same as the maintenance dose he recommended. Thus, the loading dose of Cryoprecipitated AHF has been set to be the same as the maintenance dose. g 1-2% 1, p 238, 3-5% 12. But reference 12 recommends 25-50% following major trauma. h An alternate recommendation of 500 mL q 2 d 8, p 1107 appears to be inconsistent with the half-life of Factor XIII as well as with the recommendations of references 12 and 20. i Based on the ratio of maintenance to loading doses for VWF:RCo 1, p 833. j Assuming that the product contains 1 IU/mL 3, p 22. k Each bag has about 250 mg of fibrinogen on average. 4, p 467 m Calculate volume of Profilnine SD using the estimate of 1.2 IU of factor II per IU of factor IX. 19 n Calculate volume of Profilnine SD using the estimate of 0.6 IU of factor X per IU of factor IX. 19 References 1. Colman RW et al, eds. Hemostasis and thrombosis: basic principles and clinical practice. 4th ed. Philadelphia: Lippincott Williams & Wilkins, 2001. 1578 pp. 2. Giangrande PLF. Factor XIII deficiency. Oxford Haemophilia Centre. http://www.medicine.ox.ac.uk/ohc/xiii.htm, as seen on 12/21/01. 3. American Association of Blood Banks, America's Blood Centers, American Red Cross. Circular of information for the use of human blood and blood components. http://www.aabb.org/All_About_Blood/COI/coiv2.pdf. 2000. 4. Technical manual. 13th ed. Bethesda: American Association of Blood Banks, 1999. 798 pp. 5. Blood transfusion therapy: a physician's handbook. 6th ed. Bethesda: American Association of Blood Banks, 1999. 150 pp. 6. Lechler E. Use of prothrombin complex concentrates for prophylaxis and treatment of bleeding episodes in patients with hereditary deficiency of prothrombin, factor VII, factor X, protein C, protein S, or protein Z. Thromb Res 1999;95:S39-S50. 7. Practice guidelines for blood component therapy: a report by the American Society of Anesthesiologists Task Force on Blood Component Therapy. Anesthesiology 1996;84:732-747. 8. Colman RW et al, eds. Hemostasis and thrombosis: basic principles and clinical practice. 3rd ed. Philadelphia: JB Lippincott Co, 1994. 1713 pp. 9. Fresh-Frozen Plasma, Cryoprecipitate, and Platelets Administration Practice Guidelines Development Task Force of the College of American Pathologists. Practice parameter for the use of fresh-frozen plasma, cryoprecipitate, and platelets. JAMA 1994;271:777-81. 10. Larrain AC. Deficiencia congénita en factor X de la coagulación sanguínea. Resultado exitoso del empleo de concentrados de complejo protrombinico en el control de la tendencia hemorrágica en operación cesárea en 2 embarazos [Congenital blood coagulation factor X deficiency. Successful result of the use of prothrombin concentrated complex in the control of cesarean section hemorrhage in 2 pregnancies]. Rev Med Chil 1994;122:1178–83. 11. 12. 13. 14. 15. 16. 17. 18. 19. 20. Marder VJ, Shulman NR. Clinical aspects of congenital factor VII deficiency. Am J Med 1964;37:182-94 Rodgers GM, Greenberg CS. Inherited coagulation disorders. In: Lee GR et al, eds. Wintrobe's clinical hematology. 10th ed. Baltimore: Williams & Wilkins, 1999:1682-1732. Horowitz MS, Pehta JC. SD Plasma in TTP and coagulation factor deficiencies for which no concentrates are available. Vox Sang 1998;74 Suppl 1:231-5 Saint-Raymond S, Greffe B, Carré J, et al. Attitude pratique en cas d'intervention chirurgicale chez un sujet atteint d'un déficit congénital en facteur VII [Practical approaches for surgical procedures in congenital factor VII deficiency]. Ann Fr Anesth Réanim 1989;8:518-521 Caldwell DC et al. Clinics in Obstetrics and Gynecology 1985;28(1):53-72. Cited in: Fadel HE, Krauss JS. Factor VII deficiency and pregnancy. Obstet Gynecol 1989;73(3 Pt 2):453-454 Scharrer I. Recombinant factor VIIa for patients with inhibitors to factor VIII or IX or factor VII deficiency. Haemophilia 1999; 5:253-259 USP DI. Drug information for the health care provider. Rockville, MD: United States Pharmacopeial Convention, 2002. Code of Federal Regulations, Title 21--Food and Drugs, Part 640--Additional standards for human blood and blood products. Gross, John, RN, MS; Director, Professional Services; Alpha Therapeutic Corp. E-mail to Barry Siegfried, MD; 5/10/02. Board PG et al. Factor XIII: inherited and acquired deficiency. Blood Rev 1993;7:229-242.