eMedicine Specialties > Pediatrics: General Medicine > Hematology

Hemophilia C: Follow-up

Author: Prasad Mathew, MB, BS, DCH, Director, Hemostasis and Hematology Program, Professor of Pediatrics, University of New Mexico
Coauthor(s): Paula H B Bolton-Maggs, DM, FRCP, FRCPCH, FRCPath, Consultant Haematologist, Manchester Royal Infirmary, UK
Contributor Information and Disclosures

Updated: Mar 10, 2009

Follow-up

Further Inpatient Care

  • Depending on the surgical procedure, the patient's history with other surgical procedures, and the patient's bleeding tendency if any, replacement with plasma products may be needed in the preoperative, intraoperative, and postoperative periods in patients with hemophilia C.
  • Replacement with plasma products must be coordinated with the hemophilia treatment center.
  • After a surgical procedure, patients may be discharged when complete hemostasis is achieved.

Further Outpatient Care

Annual visits to a hemophilia treatment center are recommended to provide the following care:

  • Monitoring of bleeding episodes
  • Planning for any elective surgical procedures
  • Monitoring for the development of hepatitis
  • Administering preventive immunizations as needed: In patients with severe deficiency, administer all vaccinations subcutaneously because of the risk of inducing a muscle hematoma. These patients should be vaccinated against hepatitis A virus (HAV) and hepatitis B virus (HBV) because they have or may have been exposed to plasma products as part of their treatment.
  • Continuing patient education about the bleeding condition and applying any therapeutic advances that may become available

Inpatient & Outpatient Medications

  • Advise patients to avoid taking aspirin or other nonsteroidal agents that inhibit platelet function.

Deterrence/Prevention

  • People with factor XI deficiency should be vaccinated against hepatitis B.

Complications

  • Complications of factor XI deficiency commonly involve the unpredictable nature of bleeding.
  • Patients who receive plasma products may be at risk for contracting unknown virally transmissible infections.
  • Patients may also develop inhibitors to factor XI.
  • In addition, patients who receive factor XI concentrates may be at risk for thrombotic events.

Prognosis

  • The prognosis is excellent in patients with partial deficiency without bleeding manifestations.
  • In patients with bleeding tendencies, hemorrhage and bleeding into organs may be life threatening.

Patient Education

  • Patients must be counseled about the unpredictable nature of their bleeding tendency, and they should be informed of the preparations needed before elective surgery.
  • The usual precautions regarding physical activity for individuals with a bleeding disorder apply to patients with factor XI deficiency who have a bleeding tendency. Patients should be encouraged to wear seat belts, to use protective gear (eg, bike helmets), and to avoid contact sports.
  • Patients should be advised to keep up to date with their vaccinations, especially HAV and HBV vaccinations.
  • Stress the importance of annual visits to hemophilia treatment centers.
  • Patients should receive genetic counseling with regard to their marriage partners and the potential risks to their offspring.
  • For excellent patient education resources, visit eMedicine's Blood and Lymphatic System Center. Also, see eMedicine's patient education article Hemophilia.

Miscellaneous

Medicolegal Pitfalls

  • Because factor XI concentrates are not yet available in the United States, the only potential problems are a failure to diagnose factor XI deficiency, volume overloading with the infusion of plasma products, and poor management of any hypersensitivity reactions to the infusions.
  • If factor XI concentrates are available, take care in selecting candidates for infusion, and avoid excessive dosing. See Medication.

Special Concerns

  • The unpredictable nature of bleeding is a concern, especially in patients with partial deficiency. Bleeding episodes cannot be predicted on the basis of factor XI levels alone.
  • Transmission of infections is a risk when plasma products are used for treatment.
  • Thrombotic events are a risk in patients who receive factor XI concentrates.
  • All prophylaxis vaccinations should be subcutaneously administered.
 


More on Hemophilia C

Overview: Hemophilia C
Differential Diagnoses & Workup: Hemophilia C
Treatment & Medication: Hemophilia C
Follow-up: Hemophilia C
Multimedia: Hemophilia C
References
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References

  1. Asakai R, Chung DW, Ratnoff OD, Davie EW. Factor XI (plasma thromboplastin antecedent) deficiency in Ashkenazi Jews is a bleeding disorder that can result from three types of point mutations. Proc Natl Acad Sci U S A. Oct 1989;86(20):7667-71. [Medline].

  2. Gomez K, Bolton-Maggs P. Factor XI deficiency. Haemophilia. Nov 2008;14(6):1183-9. [Medline].

  3. Bolton-Maggs PH, Peretz H, Butler R, Mountford R, Keeney S, Zacharski L. A common ancestral mutation (C128X) occurring in 11 non-Jewish families from the UK with factor XI deficiency. J Thromb Haemost. Jun 2004;2(6):918-24. [Medline].

  4. Bauduer F, Dupreuilh F, Ducout L, Marti B. Factor XI deficiency in the French Basque Country. Haemophilia. May 1999;5(3):187-90. [Medline].

  5. Bolton-Maggs PHB. Phenotype and molecular genetics of factor XI deficiency in the United Kingdom [dissertation/master's thesis]. Oxford, UK: University of Oxford; 2007.

  6. Guella I, Solda G, Spena S, Asselta R, Ghiotto R, Tenchini ML. Molecular characterization of two novel mutations causing factor XI deficiency: A splicing defect and a missense mutation responsible for a CRM+ defect. Thromb Haemost. Mar 2008;99(3):523-30. [Medline].

  7. 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. Feb 1995;73(2):194-202. [Medline].

  8. Salomon O, Steinberg DM, Koren-Morag N, Tanne D, Seligsohn U. Reduced incidence of ischemic stroke in patients with severe factor XI deficiency. Blood. Apr 15 2008;111(8):4113-7. [Medline].

  9. Gitel SN, Varon D, Schulman S, Martinowitz U. Clinical experiences of a FXI concentrate: p0ssible side effects. Throm Haemost. 1991;65:1157.

  10. Aledort LM, Forster A, Maksoud J, et al. BPL factor XI concentrate: clinical experience in the USA. Haemophilia. 1997;3:59-62.

  11. Andrew M, Paes B, Milner R, et al. Development of the human coagulation system in the full-term infant. Blood. Jul 1987;70(1):165-72. [Medline].

  12. Blat Y, Seiffert D. A renaissance for the contact system in blood coagulation?. Thromb Haemost. Mar 2008;99(3):457-60. [Medline].

  13. Bolton-Maggs PH. Bleeding problems in factor XI deficient women. Haemophilia. May 1999;5(3):155-9. [Medline].

  14. Bolton-Maggs PH. Factor XI deficiency. Baillieres Clin Haematol. Jun 1996;9(2):355-68. [Medline].

  15. Bolton-Maggs PH, Perry DJ, Chalmers EA, Parapia LA, Wilde JT, Williams MD. The rare coagulation disorders--review with guidelines for management from the United Kingdom Haemophilia Centre Doctors' Organisation. Haemophilia. Sep 2004;10(5):593-628. [Medline].

  16. Brenner B, Laor A, Lupo H, et al. Bleeding predictors in factor-XI-deficient patients. Blood Coagul Fibrinolysis. Nov 1997;8(8):511-5. [Medline].

  17. Broze GJ, Gailani D. The role of factor XI in coagulation. Thromb Haemost. Jul 1 1993;70(1):72-4. [Medline].

  18. Gailani D, Schmidt A, Sun MF, Bolton-Maggs PH, Bajaj SP. A cross-reactive material positive variant of coagulation factor XI (FXIP520L) with a catalytic defect. J Thromb Haemost. Apr 2007;5(4):781-7. [Medline].

  19. Imanaka Y, McVey JH, Nichimura T, et al. Identification and characterization of mutations in factor XI gene of non-Jewish factor XI deficient patients. Thromb Haemost. 1993;69:752-60.

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

  21. Kadir RA, Economides DL, Sabin CA, et al. Frequency of inherited bleeding disorders in women with menorrhagia. Lancet. Feb 14 1998;351(9101):485-9. [Medline].

  22. Kadir RA, Kingman CE, Chi C, O'connell NM, Riddell A, Lee CA, et al. Screening for factor XI deficiency amongst pregnant women of Ashkenazi Jewish origin. Haemophilia. Nov 2006;12(6):625-8. [Medline].

  23. Kato A, Asakai R, Davie EW, Aoki N. Factor XI gene (F11) is located on the distal end of the long arm of human chromosome 4. Cytogenet Cell Genet. 1989;52(1-2):77-8. [Medline].

  24. Mannhalter C, Hellstern P, Deutsch E. Identification of a defective factor XI cross-reacting material in a factor XI-deficient patient. Blood. Jul 1987;70(1):31-7. [Medline].

  25. Peter MK, Meili EO, von Felton A. Factor XI deficiency: additional hemostatic defects are present in patients with bleeding tendency. Thromb Haemost. 1995;73:1442.

  26. Pugh RE, McVey JH, Tuddenham EG, Hancock JF. Six point mutations that cause factor XI deficiency. Blood. Mar 15 1995;85(6):1509-16. [Medline].

  27. Ragni MV, Sinha D, Seaman F, et al. Comparison of bleeding tendency, factor XI coagulant activity, and factor XI antigen in 25 factor XI-deficient kindreds. Blood. Mar 1985;65(3):719-24. [Medline].

  28. Rosenthal RL, Dreskin OH, Rosenthal N. Plasma thromboplastin antecedent (PTA) deficiency; clinical, coagulation, therapeutic and hereditary aspects of a new hemophilia-like disease. Blood. Feb 1955;10(2):120-31. [Medline].

  29. Salomon O, Seligsohn U. New observations on factor XI deficiency. Haemophilia. Oct 2004;10 Suppl 4:184-7.

  30. 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. Sep 2006;12(5):490-3. [Medline].

  31. Salomon O, Zivelin A, Livnat T, et al. Prevalence, causes, and characterization of factor XI inhibitors in patients with inherited factor XI deficiency. Blood. Jun 15 2003;101(12):4783-8. [Medline].

  32. Seligsohn U. Factor XI deficiency. Thromb Haemost. Jul 1 1993;70(1):68-71. [Medline].

  33. Seligsohn U, Peretz H. Molecular genetics aspects of factor XI deficiency and Glanzmann thrombasthenia. Haemostasis. Mar-Apr 1994;24(2):81-5. [Medline].

  34. Zivelin A, Ogawa T, Bulvik S, Landau M, Toomey JR, Lane J. 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. Oct 2004;2(10):1782-9. [Medline].

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Further Reading

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Keywords

hemophilia C, plasma thromboplastin antecedent deficiency, factor XI deficiency, factor XIC, factor XIc, clotting, deficiency of factor XI, clotting activity, blood coagulation, thrombin-activatable fibrinolysis inhibitor, TAFI, bleeding tendency, excessive bleeding, ischemic stroke, von Willebrand disease, menorrhagia, tonsillectomy, massive hemothorax, cerebral hemorrhage, subarachnoid hemorrhage, spinal epidural hematoma, systemic lupus erythematosus, Noonan syndrome

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Contributor Information and Disclosures

Author

Prasad Mathew, MB, BS, DCH, Director, Hemostasis and Hematology Program, Professor of Pediatrics, University of New Mexico
Prasad Mathew, MB, BS, DCH is a member of the following medical societies: American Society of Hematology
Disclosure: Nothing to disclose.

Coauthor(s)

Paula H B Bolton-Maggs, DM, FRCP, FRCPCH, FRCPath, Consultant Haematologist, Manchester Royal Infirmary, UK
Paula H B Bolton-Maggs, DM, FRCP, FRCPCH, FRCPath is a member of the following medical societies: American Society of Hematology and International Society on Thrombosis and Haemostasis
Disclosure: no financial interest None None

Medical Editor

Gary R Jones, MD, Associate Medical Director, Clinical Development, Berlex Laboratories
Gary R Jones, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Pediatric Hematology/Oncology, and Western Society for Pediatric Research
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Gary D Crouch, MD, Program Director of Pediatric Hematology-Oncology Fellowship, Department of Pediatrics, Associate Professor, Uniformed Services University of the Health Sciences
Gary D Crouch, MD is a member of the following medical societies: American Academy of Pediatrics and American Society of Hematology
Disclosure: Nothing to disclose.

CME Editor

Samuel Gross, MD, Professor Emeritus, Department of Pediatrics, University of Florida; Clinical Professor, Department of Pediatrics, University of North Carolina; Adjunct Professor, Department of Pediatrics, Duke University
Samuel Gross, MD is a member of the following medical societies: American Association for Cancer Research, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, American Society of Hematology, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Chief Editor

Robert J Arceci, MD, PhD, King Fahd Professor of Pediatric Oncology, Department of Oncology, Division of Pediatric Oncology, Johns Hopkins University School of Medicine
Robert J Arceci, MD, PhD is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, and American Society of Pediatric Hematology/Oncology
Disclosure: Nothing to disclose.

 
 
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