Factor XI Deficiency Medication
- Author: Jamie E Siegel, MD; Chief Editor: Srikanth Nagalla, MBBS, MS, FACP more...
To achieve a FXI level of 50%, a patient needs to have half of their plasma volume replaced.
Directed-donor fresh frozen plasma can be arranged for elective procedures, but a large volume of plasma will need to be stored to transfuse for the required 7-14 days after surgery. One study has demonstrated that solvent detergent fresh frozen plasma has a half-life of 45 hours, while a second study has shown that FXI is decreased in the product.
Plasma is the fluid compartment of blood containing the soluble clotting factors. Octaplas is a solvent detergent treated, pooled FFP.
May consider prophylactic administration with antifibrinolytics prior to minor procedures (eg, dental).
Inhibits fibrinolysis via inhibition of plasminogen activator substances and, to a lesser degree, through antiplasmin activity. Main problem is that the thrombi that form during treatment are not lysed, and effectiveness is uncertain.
Alternative to aminocaproic acid. Inhibits fibrinolysis by displacing plasminogen from fibrin.
Choi SH, Smith SA, Morrissey JH. Polyphosphate is a cofactor for the activation of factor XI by thrombin. Blood. 2011 Dec 22. 118(26):6963-70. [Medline].
Rugeri L, Quélin F, Chatard B, De Mazancourt P, Negrier C, Dargaud Y. Thrombin generation in patients with factor XI deficiency and clinical bleeding risk. Haemophilia. 2010 Sep 1. 16(5):771-7. [Medline].
Saunders RE, Shiltagh N, Gomez K, et al. Structural analysis of eight novel and 112 previously reported missense mutations in the interactive FXI mutation database reveals new insight on FXI deficiency. Thromb Haemost. 2009 Aug. 102(2):287-301. [Medline].
Qu Y, Nie X, Yang Z, Yin H, Pang Y, Dong P, et al. The prevalence of hemophilia in mainland China: a systematic review and meta-analysis. Southeast Asian J Trop Med Public Health. 2014 Mar. 45(2):455-66. [Medline].
Knol HM, Mulder AB, Bogchelman DH, Kluin-Nelemans HC, van der Zee AG, Meijer K. The prevalence of underlying bleeding disorders in patients with heavy menstrual bleeding with and without gynecologic abnormalities. Am J Obstet Gynecol. 2013 Sep. 209(3):202.e1-7. [Medline].
Guéguen P, Galinat H, Blouch MT, Bridey F, Duchemin J, Le Gal G, et al. Biological determinants of bleeding in patients with heterozygous factor XI deficiency. Br J Haematol. 2012 Jan. 156(2):245-51. [Medline].
Asakai R, Chung DW, Davie EW, Seligsohn U. Factor XI deficiency in Ashkenazi Jews in Israel. N Engl J Med. 1991 Jul 18. 325(3):153-8. [Medline].
Azzi A, De Santis R, Morfini M, et al. TT virus contaminates first-generation recombinant factor VIII concentrates. Blood. 2001 Oct 15. 98(8):2571-3. [Medline].
Bauduer F, Bendriss P, Freyburger G, et al. Use of desmopressin for prophylaxis of surgical bleeding in factor XI- deficient patients. Acta Haematol. 1998. 99(1):52-3. [Medline].
Berliner S, Horowitz I, Martinowitz U, et al. Dental surgery in patients with severe factor XI deficiency without plasma replacement. Blood Coagul Fibrinolysis. 1992 Aug. 3(4):465-8. [Medline].
Bolton-Maggs PH. Factor XI deficiency and its management. World Federation of Hemophilia Monograph. 1999:16.
Bolton-Maggs PH. Factor XI deficiency and its management. Haemophilia. 2000 Jul. 6 Suppl 1:100-9. [Medline].
Bolton-Maggs PH, Patterson DA, Wensley RT, Tuddenham EG. Definition of the bleeding tendency in factor XI-deficient kindreds--a clinical and laboratory study. Thromb Haemost. 1995 Feb. 73(2):194-202. [Medline].
Bolton-Maggs PH, Peretz H, Butler R, et al. A common ancestral mutation (C128X) occurring in 11 non-Jewish families from the UK with factor XI deficiency. J Thromb Haemost. 2004 Jun. 2(6):918-24.
Bonnar J, Sheppard BL. Treatment of menorrhagia during menstruation: randomised controlled trial of ethamsylate, mefenamic acid, and tranexamic acid. BMJ. 1996 Sep 7. 313(7057):579-82. [Medline].
Castaman G, Ruggeri M, Rodeghiero F. Clinical usefulness of desmopressin for prevention of surgical bleeding in patients with symptomatic heterozygous factor XI deficiency. Br J Haematol. 1996 Jul. 94(1):168-70. [Medline].
Clarkson K, Rosenfeld B, Fair J, et al. Factor XI deficiency acquired by liver transplantation. Ann Intern Med. 1991 Dec 1. 115(11):877-9. [Medline].
Coukos G, Rubin SC. Gene therapy for ovarian cancer. Oncology (Huntingt). 2001 Sep. 15(9):1197-204, 1207; discussion 1207-8. [Medline].
Di Bisceglie AM. SEN and sensibility: interactions between newly discovered and other hepatitis viruses?. Lancet. 2001 Dec 8. 358(9297):1925-6. [Medline].
Dzik WH, Arkin CF, Jenkins RL. Transfer of congenital factor XI deficiency from a donor to a recipient by liver transplantation. N Engl J Med. 1987 May 7. 316(19):1217-8. [Medline].
Franchini M, Manzato F, Salvagno GL, Montagnana M, Lippi G. The use of desmopressin in congenital factor XI deficiency: a systematic review. Ann Hematol. 2009 Jul 17. epub ahead of print. [Medline].
Hancock JF, Wieland K, Pugh RE, et al. A molecular genetic study of factor XI deficiency. Blood. 1991 May 1. 77(9):1942-8. [Medline].
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. 241-265. [Medline].
Kadir RA, Economides DL, Lee CA. Factor XI deficiency in women. Am J Hematol. 1999 Jan. 60(1):48-54. [Medline].
Kravtsov DV, Monahan PE, Gailani D, et al. A classification system for cross-reactive material-negative factor XI deficiency. Blood. 2005 Jun 15. 105(12):4671-3.
Martincic D, Zimmerman SA, Ware RE, et al. Identification of mutations and polymorphisms in the factor XI genes of an African American family by dideoxyfingerprinting. Blood. 1998 Nov 1. 92(9):3309-17. [Medline].
MediView Express. Recombinant therapy enhances safety andquality of life for hemophilia patients. Paper presented at: 53rd Annual Meeting of the National Hemophilia Foundation. November 16, 2001. Nashville, Tennessee.
Peretz H, Mulai A, Usher S, et al. The two common mutations causing factor XI deficiency in Jews stem from distinct founders: one of ancient Middle Eastern origin and another of more recent European origin. Blood. 1997 Oct 1. 90(7):2654-9. [Medline].
Rigas B, Hasan I, Rehman R, et al. Effect on treatment outcome of coinfection with SEN viruses in patients with hepatitis C. Lancet. 2001 Dec 8. 358(9297):1961-2. [Medline].
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. 82:171-4.
Salomon O, Seligsohn U. New observations on factor XI deficiency. Haemophilia. 2004 Oct. 10 Suppl 4:184-7.
Salomon O, Zivelin A, Livnat T, et al. Prevalence, causes, and characterization of factor XI inhibitors in patients with inherited factor XI deficiency. Blood. 2003 Jun 15. 101(12):4783-8. [Medline].
Saunders RE, O''Connell NM, Lee CA, et al. Factor XI deficiency database: an interactive web database of mutations, phenotypes, and structural analysis tools. Hum Mutat. 2005 Sep. 26(3):192-8.
Seligsohn U. Factor XI deficiency in humans. J Thromb Haemost. 2009 Jul. 7 suppl 1:84-7. [Medline].
Seligsohn U. The activated partial thromboplastin time and factor XI deficiency. Ann Intern Med. 1986 Nov. 105(5):805. [Medline].
Seligsohn U, White GC. Inherited deficiencies of coagulation factors II, V, VII, XI, and XIII and the combined deficiencies of factors V and VIII and of the vitamin K-dependent factors. In: Beutler E, Lichtman MA,Coller BS, et al, eds. Williams Hematology. McGraw-Hill. 2001:1617-38.
Siegel JE, Kouides PA. Menorrhagia from a haematologist''s point of view. Part II: management. Haemophilia. 2002 May. 8(3):339-47. [Medline].
Sun MF, Baglia FA, Ho D, et al. Defective binding of factor XI-N248 to activated human platelets. Blood. 2001 Jul 1. 98(1):125-9. [Medline].
Walsh P. Factor XI. In: Colman RW, Hirsh J, Marder VJ, et al, eds. Hemostasis and Thrombosis: Basic Principles and Clinical Practice. 4th ed. Lippincott Williams & Wilkins. 1987.
Zivelin A, Bauduer F, Ducout L, et al. Factor XI deficiency in French Basques is caused predominantly by an ancestral Cys38Arg mutation in the factor XI gene. Blood. 2002 Apr 1. 99(7):2448-54. [Medline].
Zivelin A, Ogawa T, Bulvik S, et al. Severe factor XI deficiency caused by a Gly555 to Glu mutation (factor XI-Glu555): a cross-reactive material positive variant defective in factor IX activation. J Thromb Haemost. 2004 Oct. 2(10):1782-9.