eMedicine Specialties > Hematology > Coagulation, Hemostasis, and Disorders

Factor X: Follow-up

Author: Christopher J Steen, MD, Staff Physician, Department of Dermatology, University of Medicine and Dentistry of New Jersey, New Jersey Medical School
Coauthor(s): Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School; Pere Gascon, MD, PhD, Professor and Director, Division of Medical Oncology, Institute of Hematology and Medical Oncology, IDIBAPS, University of Barcelona Faculty of Medicine, Spain
Contributor Information and Disclosures

Updated: Dec 1, 2008

Follow-up

Prognosis

  • The prognosis for patients with factor X deficiency depends on the etiology and severity of the disease. Although acquired factor X deficiency may be eliminated by treating the underlying cause, the congenital form of the disease is lifelong and is among the most severe clotting factor disorders. In general, patients with very low levels of functional factor X have a greater tendency to hemorrhage and face a greater risk of life-threatening complications.

Patient Education

Miscellaneous

Medicolegal Pitfalls

  • Misdiagnosis of the type of factor disorder
  • Failure to diagnose the presence of factor inhibitors
  • Failure to recognize an underlying disease process
 


More on Factor X

Overview: Factor X
Differential Diagnoses & Workup: Factor X
Treatment & Medication: Factor X
Follow-up: Factor X
References
Further Reading

References

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  2. Hougie C, Barrow EM, Graham JB. Stuart clotting defect. I. Segregation of an hereditary hemorrhagic state from the heterogeneous group heretofore called stable factor (SPCA, proconvertin, factor VII) deficiency. J Clin Invest. Mar 1957;36(3):485-96. [Medline][Full Text].

  3. Uprichard J, Perry DJ. Factor X deficiency. Blood Rev. Jun 2002;16(2):97-110. [Medline].

  4. Pfeiffer RA, Ott R, Gilgenkrantz S, Alexandre P. Deficiency of coagulation factors VII and X associated with deletion of a chromosome 13 (q34). Evidence from two cases with 46,XY,t(13;Y)(q11;q34). Hum Genet. 1982;62(4):358-60. [Medline].

  5. Pfeiffer RA, Ott R, Taben KD. Clotting factors VII and X as useful markers of terminal deletion of chromosome 13. Hum Genet. 1985;69(2):192. [Medline].

  6. Ott R, Pfeiffer RA. Evidence that activities of coagulation factors VII and X are linked to chromosome 13 (q34). Hum Hered. 1984;34(2):123-6. [Medline].

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  9. Peyvandi F, Menegatti M, Santagostino E, et al. Gene mutations and three-dimensional structural analysis in 13 families with severe factor X deficiency. Br J Haematol. Jun 2002;117(3):685-92. [Medline].

  10. Morishita E, Yamaguchi K, Asakura H, et al. One missense mutation in the factor X gene causing factor X deficiency--factor X Kanazawa. Int J Hematol. Apr 2001;73(3):390-2. [Medline].

  11. Isshiki I, Favier R, Moriki T, et al. Genetic analysis of hereditary factor X deficiency in a French patient of Sri Lankan ancestry: in vitro expression study identified Gly366Ser substitution as the molecular basis of the dysfunctional factor X. Blood Coagul Fibrinolysis. Jan 2005;16(1):9-16. [Medline].

  12. Wang WB, Fu QH, Zhou RF, et al. Molecular characterization of two novel mutations causing factor X deficiency in a Chinese pedigree. Haemophilia. Jan 2005;11(1):31-7. [Medline].

  13. Jayandharan G, Viswabandya A, Baidya S, et al. Six novel mutations including triple heterozygosity for Phe31Ser, 514delT and 516T-->G factor X gene mutations are responsible for congenital factor X deficiency in patients of Nepali and Indian origin. J Thromb Haemost. Jul 2005;3(7):1482-7. [Medline][Full Text].

  14. Shinohara K, Adachi M, Matsui K, et al. A case of factor X (FX) deficiency due to novel mutation V196M, FX Hofu. Int J Hematol. Apr 2008;87(3):256-9. [Medline].

  15. Bereczky Z, Bardos H, Komaromi I, et al. Factor X Debrecen: Gly204Arg mutation in factor X causes the synthesis of a non-secretable protein and severe factor X deficiency. Haematologica. Feb 2008;93(2):299-302. [Medline][Full Text].

  16. Al-Hilali A, Wulff K, Abdel-Razeq H, et al. Analysis of the novel factor X gene mutation Glu51Lys in two families with factor X-Riyadh anomaly. Thromb Haemost. Apr 2007;97(4):542-5. [Medline].

  17. Todd T, Perry DJ, Hayman E, et al. Severe factor X deficiency due to a homozygous mutation (Cys364Arg) that disrupts a disulphide bond in the catalytic domain. Haemophilia. Nov 2006;12(6):621-4. [Medline].

  18. Zama T, Murata M, Watanabe R, et al. A family with hereditary factor X deficiency with a point mutation Gla32 to Gln in the Gla domain (factor X Tokyo). Br J Haematol. Sep 1999;106(3):809-11. [Medline].

  19. Bezeaud A, Miyata T, Helley D, et al. Functional consequences of the Ser334-->Pro mutation in a human factor X variant (factor X Marseille). Eur J Biochem. Nov 15 1995;234(1):140-7. [Medline][Full Text].

  20. Choufani EB, Sanchorawala V, Ernst T, et al. Acquired factor X deficiency in patients with amyloid light-chain amyloidosis: incidence, bleeding manifestations, and response to high-dose chemotherapy. Blood. Mar 15 2001;97(6):1885-7. [Medline][Full Text].

  21. Furie B, Voo L, McAdam KP, Furie BC. Mechanism of factor X deficiency in systemic amyloidosis. N Engl J Med. Apr 2 1981;304(14):827-30. [Medline].

  22. Perez Martinez J, Llamas F, Lopez Montes A, et al. [Primary amyloidosis associated to severe factor X deficiency] [Spanish]. Nefrologia. 2004;24(5):493-8. [Medline].

  23. Schwarzinger I, Stain-Kos M, Bettelheim P, et al. Recurrent, isolated factor X deficiency in myeloma: repeated normalization of factor X levels after cytostatic chemotherapy followed by late treatment failure associated with the development of systemic amyloidosis. Thromb Haemost. Dec 7 1992;68(6):648-51. [Medline].

  24. Peuscher FW, van Aken WG, van Mourik JA, et al. Acquired, transient factor X (Stuart factor) deficiency in patient with mycoplasma pneumonial infection. Scand J Haematol. Oct 1979;23(4):257-64. [Medline].

  25. Ashrani AA, Aysola A, Al-Khatib H, Nichols WL, Key NS. Lupus anticoagulant associated with transient severe factor X deficiency: a report of two patients presenting with major bleeding complications. Br J Haematol. May 2003;121(4):639-42. [Medline].

  26. Gallais V, Bredoux H, le Roux G, Laroche L. Acquired and transient factor X deficiency associated with sodium valproate treatment. Eur J Haematol. Oct 1996;57(4):330. [Medline].

  27. Mulhare PE, Tracy PB, Golden EA, Branda RF, Bovill EG. A case of acquired factor X deficiency with in vivo and in vitro evidence of inhibitor activity directed against factor X. Am J Clin Pathol. Aug 1991;96(2):196-200. [Medline].

  28. Ness PM, Hymas PG, Gesme D, Perkins HA. An unusual factor-X inhibitor in leprosy. Am J Hematol. 1980;8(4):397-402. [Medline].

  29. Matsunaga AT, Shafer FE. An acquired inhibitor to factor X in a pediatric patient with extensive burns. J Pediatr Hematol Oncol. May 1996;18(2):223-6. [Medline].

  30. Israels SJ, Leaker MT. Acquired inhibitors to factors V and X after exposure to topical thrombin: interference with monitoring of low molecular weight heparin and warfarin. J Pediatr. Sep 1997;131(3):480-3. [Medline].

  31. Nora RE, Bell WR, Noe DA, Sholar PW. Novel factor X deficiency. Normal partial thromboplastin time and associated spindle cell thymoma. Am J Med. Jul 1985;79(1):122-6. [Medline].

  32. Caimi MT, Redaelli R, Cattaneo D, et al. Acquired selective factor X deficiency in acute nonlymphocytic leukemia. Am J Hematol. Jan 1991;36(1):65-6. [Medline].

  33. Peyvandi F, Mannucci PM. Rare coagulation disorders. Thromb Haemost. Oct 1999;82(4):1207-14. [Medline].

  34. Citak A, Ucsel R, Karabocuoglu M, Unuvar A, Uzel N. A rare cause of intracranial hemorrhage: factor X deficiency. Pediatr Emerg Care. Oct 2001;17(5):349-50. [Medline].

  35. Young TM, Chitnavis BP, Swallow EB, Arya R, Vadher BD. Intracerebral haemorrhage in an adult due to transient factor X deficiency. J R Soc Med. Jul 2003;96(7):355-6. [Medline][Full Text].

  36. Herrmann FH, Navarette M, Salazar-Sanchez L, et al. Homozygous Factor X gene mutations Gly380Arg and Tyr163delAT are associated with perinatal intracranial hemorrhage. J Pediatr. Jan 2005;146(1):128-30. [Medline].

  37. Greipp PR, Kyle RA, Bowie EJ. Factor X deficiency in primary amyloidosis: resolution after splenectomy. N Engl J Med. Nov 8 1979;301(19):1050-1. [Medline].

  38. Rosenstein ED, Itzkowitz SH, Penziner AS, Cohen JI, Mornaghi RA. Resolution of factor X deficiency in primary amyloidosis following splenectomy. Arch Intern Med. Mar 1983;143(3):597-9. [Medline].

  39. McMahon C, Smith J, Goonan C, Byrne M, Smith OP. The role of primary prophylactic factor replacement therapy in children with severe factor X deficiency. Br J Haematol. Dec 2002;119(3):789-91. [Medline].

Keywords

factor X, factor X deficiency, FX, FX deficiency, Stuart factor, Prower factor, Stuart-Prower factor, autoprothrombin III, thrombokinase, blood coagulation factor X, coagulation factor X, vitamin K deficiency, liver disease, hepatic disease, blood disorder, factor disorder, coagulation disorder, clotting disorder, bleeding disorder, blood factor deficiency, factor deficiency, easy bruising, hematuria, soft-tissue hemorrhages, hemarthroses, recurrent epistaxis, menorrhagia, congenital factor X deficiency, acquired factor X deficiency

Contributor Information and Disclosures

Author

Christopher J Steen, MD, Staff Physician, Department of Dermatology, University of Medicine and Dentistry of New Jersey, New Jersey Medical School
Christopher J Steen, MD is a member of the following medical societies: Alpha Omega Alpha and Sigma Xi
Disclosure: Nothing to disclose.

Coauthor(s)

Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi
Disclosure: Nothing to disclose.

Pere Gascon, MD, PhD, Professor and Director, Division of Medical Oncology, Institute of Hematology and Medical Oncology, IDIBAPS, University of Barcelona Faculty of Medicine, Spain
Pere Gascon, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, New York Academy of Medicine, New York Academy of Sciences, and Sigma Xi
Disclosure: Nothing to disclose.

Medical Editor

Paul Schick, MD, Emeritus Professor, Department of Internal Medicine, Thomas Jefferson University Medical College; Research Professor, Department of Internal Medicine, Drexel University College of Medicine; Adjunct Professor of Medicine, Lankenau Hospital, Wynnewood, PA
Paul Schick, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Society of Hematology, International Society on Thrombosis and Haemostasis, and New York Academy of Sciences
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Ronald A Sacher, MB, BCh, MD, FRCPC, Professor, Internal Medicine and Pathology, Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center
Ronald A Sacher, MB, BCh, MD, FRCPC is a member of the following medical societies: American Society of Hematology
Disclosure: Glaxo Smith Kline Honoraria Speaking and teaching; Talecris Honoraria Board membership

CME Editor

Rebecca J Schmidt, DO, FACP, FASN, Professor of Medicine, Section Chief, Department of Medicine, Section of Nephrology, West Virginia University School of Medicine
Rebecca J Schmidt, DO, FACP, FASN is a member of the following medical societies: American College of Osteopathic Internists, American College of Physicians, American Medical Association, American Society of Nephrology, International Society of Nephrology, National Kidney Foundation, Renal Physicians Association, and West Virginia State Medical Association
Disclosure: Abbott Grant/research funds Speaking and teaching; Genzyme Honoraria Consulting; Amgen Honoraria Speaking and teaching; Ortho Biotech Honoraria Speaking and teaching

Chief Editor

Emmanuel C Besa, MD, Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Thomas Jefferson University
Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, and New York Academy of Sciences
Disclosure: Nothing to disclose.

 
 
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