Factor XI Deficiency Follow-up

1. Wheeler AP, Gailani D. Why factor XI deficiency is a clinical concern. Expert Rev Hematol. 2016 Jul. 9 (7):629-37. [Medline]. [Full Text].

2. Kadir RA, Economides DL, Lee CA. Factor XI deficiency in women. Am J Hematol. 1999 Jan. 60(1):48-54. [Medline]. [Full Text].

3. 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.

4. 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].

5. 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].

6. 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].

7. 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].

8. Kravtsov DV, Monahan PE, Gailani D. A classification system for cross-reactive material-negative factor XI deficiency. Blood. 2005 Jun 15. 105 (12):4671-3. [Medline]. [Full Text].

9. 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].

10. 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].

11. Seligsohn U. Factor XI deficiency in humans. J Thromb Haemost. 2009 Jul. 7 suppl 1:84-7. [Medline].

12. 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].

13. 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].

14. 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].

15. 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].

16. 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].

17. Gidley GN, Holle LA, Burthem J, Bolton-Maggs PHB, Lin FC, Wolberg AS. Abnormal plasma clot formation and fibrinolysis reveal bleeding tendency in patients with partial factor XI deficiency. Blood Adv. 2018 May 22. 2 (10):1076-1088. [Medline]. [Full Text].

18. Gerber GF, Klute KA, Chapin J, Bussel J, DeSancho MT. Peri- and Postpartum Management of Patients With Factor XI Deficiency. Clin Appl Thromb Hemost. 2019 Jan-Dec. 25:1076029619880262. [Medline].

19. 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].

20. Salomon O, Steinberg DM, Seligshon U. Variable bleeding manifestations characterize different types of surgery in patients with severe factor XI deficiency enabling parsimonious use of replacement therapy. Haemophilia. 2006 Sep. 12 (5):490-3. [Medline].

21. 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].

22. 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].

23. 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].

24. Salomon O, Budnik I, Avishai E, Tamarin I, Bashari D, Dardik R, et al. Single Low Dose of rFVIIa Combined with Antifibrinolytic Agent is a Simple and Safe Treatment for Factor XI-Deficient Patients undergoing Surgery. Thromb Haemost. 2019 Sep 7. [Medline].

25. Minami H, Nogami K, Yada K, Ogiwara K, Furukawa S, Soeda T, et al. Emicizumab, the bispecific antibody to factors IX/IXa and X/Xa, potentiates coagulation function in factor XI-deficient plasma in vitro. J Thromb Haemost. 2019 Jan. 17 (1):126-137. [Medline].

Jamie E Siegel, MD Director, Cardeza Foundation Hemophilia Treatment Center, Thomas Jefferson University

Jamie E Siegel, MD is a member of the following medical societies: American College of Physicians, American Society for Clinical Pathology, American Society of Hematology, International Society on Thrombosis and Haemostasis

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Ronald A Sacher, MBBCh, FRCPC, DTM&H Professor of Internal Medicine and Pathology, Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center

Ronald A Sacher, MBBCh, FRCPC, DTM&H is a member of the following medical societies: American Association for the Advancement of Science, American Association of Blood Banks, American Clinical and Climatological Association, American Society for Clinical Pathology, American Society of Hematology, College of American Pathologists, International Society of Blood Transfusion, International Society on Thrombosis and Haemostasis, Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Srikanth Nagalla, MBBS, MS, FACP Chief of Benign Hematology, Miami Cancer Institute

Srikanth Nagalla, MBBS, MS, FACP is a member of the following medical societies: American Society of Hematology, Association of Specialty Professors

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Alexion; Alnylam; Kedrion; Sanofi .

Paul Schick, MD † Emeritus Professor, Department of Internal Medicine, Jefferson Medical College of Thomas Jefferson University; Research Professor, Department of Internal Medicine, Drexel University College of Medicine; Adjunct Professor of Medicine, Lankenau Hospital

Paul Schick, MD is a member of the following medical societies: American College of Physicians, American Society of Hematology

Disclosure: Nothing to disclose.

• Print this section
• Print the entire contents of
• Print the entire contents of article