Hemophilia C Treatment & Management
- Author: Paula H B Bolton-Maggs, DM, FRCP, FRCPCH, FRCPath; Chief Editor: Robert J Arceci, MD, PhD more...
Approach Considerations
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.
Thrombotic events are a risk in some patients who receive factor XI concentrates.
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 and hepatitis B virus, because they have or may have been exposed to plasma products as part of their treatment.
Go to Acquired Hemophilia, Hemophilia A, and Hemophilia B for complete information on these topics.
Activity
Advise patients with factor XI deficiency to participate only in age-appropriate activities. Physical activity precautions also apply to patients with factor XI deficiency who have a bleeding tendency. Advise them against participating in contact sports if the patient has severe disease.
Consultations
Consult a pediatric or adult hematologist when the patient presents with excessive bleeding or when a preoperative laboratory evaluation reveals a prolonged aPTT.
Managing Bleeding in Surgical Procedures
Treatment of patients with factor XI deficiency is a challenge. Patients with severe deficiency are clearly and commonly at risk of bleeding from surgical procedures. Bleeding in these patients can start at the time of injury, or it can be delayed for several hours; it may persist until specific treatment is administered or it can stop on its own.
Bleeding is much more likely in relation to surgery in areas of high fibrinolytic activity and is less common in other procedures.
Patients with severe factor XI deficiency usually require replacement therapy before they undergo a surgical procedure, even if they have never bled after surgery before. Patients with partial deficiency can also have bleeding episodes, and plans for replacement therapy depend on previous history and the site of surgery.
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.
The management of the patient should be discussed jointly between surgeon, hematologist and anesthesiologist, and a management plan set out in writing. Generally, the use of nonsteroidal anti-inflammatory drugs (NSAIDs) should be avoided.
Replacement with plasma products must be coordinated with the hemophilia treatment center.
After a surgical procedure, discharge depends on the type of surgery and how long replacement therapy is needed, which may be 5-7 days after major surgery.
The basic principle of management consists of altering the balance between bleeding and clotting. Therapy consists of replacing the deficient factor and using other measures, such as fibrin glue and antifibrinolytics.
Long-Term Monitoring
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
Continuing patient education about the bleeding condition and applying any therapeutic advances that may become available.
Brenner B, Laor A, Lupo H, Zivelin A, Lanir N, Seligsohn U. Bleeding predictors in factor-XI-deficient patients. Blood Coagul Fibrinolysis. Nov 1997;8(8):511-5. [Medline].
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].
Guella I, Soldà G, Spena S, Asselta R, Ghiotto R, Tenchini ML, et al. 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].
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].
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]. [Full Text].
Gomez K, Bolton-Maggs P. Factor XI deficiency. Haemophilia. Nov 2008;14(6):1183-9. [Medline].
Bolton-Maggs PH, Peretz H, Butler R, Mountford R, Keeney S, Zacharski L, et al. 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].
Bauduer F, Dupreuilh F, Ducout L, Marti B. Factor XI deficiency in the French Basque Country. Haemophilia. May 1999;5(3):187-90. [Medline].
Aledort LM, Forster A, Maksoud J, et al. BPL factor XI concentrate: clinical experience in the USA. Haemophilia. 1997;3:59-62.
Gitel SN, Varon D, Schulman S, Martinowitz U. Clinical experiences of a FXI concentrate: possible side effects. Throm Haemost. 1991;65:1157.
Print
