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Factor XI Deficiency Treatment & Management

  • Author: Jamie E Siegel, MD; Chief Editor: Srikanth Nagalla, MBBS, MS, FACP  more...
 
Updated: May 13, 2016
 

Approach Considerations

Patients with factor XI (FXI) deficiency do not need treatment or prophylaxis for routine functions or activities. They do need treatment for dental extractions and surgery. Expectant treatment of a pregnant woman is controversial if a cesarean delivery is not planned. Treatment of FXI deficiency is determined by the intervention planned.

Fresh frozen plasma (Octaplas) has been the most available source of FXI. The recovery of FXI function from plasma is excellent, and the half-life is 40-80 hours.

Dental procedures have been performed safely with the use of factor replacement. Administration of antifibrinolytics alone was attempted when patients with FXI deficiency were recognized to be more likely to bleed in areas of high fibrinolysis. Patients since have been treated successfully with the use of tranexamic acid alone in preparation for dental extraction. The treatment is begun before the procedure and continued for an additional week.

Invasive surgical procedures often require fresh frozen plasma replacement. This should be continued for 7-14 days after surgery. Remember that the half-life of FXI is approximately 52 hours (2 d).

Pregnant women will need fresh frozen plasma if cesarean delivery is planned. Peripartum treatment of women with FXI deficiency is controversial. One group treats patients to maintain FXI levels above 50% during labor and then continues treatment for 3-4 days after vaginal delivery and 7 days after cesarean delivery. This is recommended because of the high incidence of postpartum hemorrhage. The recommendation to treat expectantly must be understood in the context of the known variability of bleeding manifestations based on patient history and FXI level, as well as the unpredictable risk of exposure to blood-borne pathogens with the use of fresh frozen plasma.

The use of desmopressin, a vasopressin analog, used for patients with factor VIII deficiency, von Willebrand disease, and platelet function abnormalities, has been tried in a handful of patients with FXI deficiency. In the patients reported, three of whom had baseline FXI levels ranging from 34-45%, factor level increased from 12% to 23%. In one patient with severe (< 1%) FXI deficiency, the level did not increase. The four patients presented in these published reports had no surgical bleeding. The true benefit of this treatment is unclear, and it is not recommended for major surgical procedures.

Antifibrinolytic therapy has been used in the treatment of women with FXI deficiency and menorrhagia.

Treatment of patients with acquired antibodies to FXI has not been standardized because of the infrequency of this occurrence. Successful treatment has been reported during invasive procedures with the use of plasma, prothrombin complex concentrates, and recombinant activated factor VII. Reports also exist of patients with inhibitors who have no spontaneous bleeds.

Unless needed for another medical indication, aspirin products should be avoided by patients with FXI deficiency.

Immunization with hepatitis A virus and hepatitis B virus vaccines is recommended prior to planned surgery and plasma product replacement. Consultation with a hematologist is recommended.

 
 
Contributor Information and Disclosures
Author

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.

Specialty Editor Board

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, MB, BCh, FRCPC, DTM&H Professor, Internal Medicine and Pathology, Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center

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

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: GSK Pharmaceuticals,Alexion,Johnson & Johnson Talecris,,Grifols<br/>Received honoraria from all the above companies for speaking and teaching.

Chief Editor

Srikanth Nagalla, MBBS, MS, FACP Director, Clinical Hematology, Cardeza Foundation for Hematologic Research; Assistant Professor of Medicine, Division of Hematology, Associate Program Director, Hematology/Medical Oncology Fellowship, Assistant Program Director, Internal Medicine Residency, Jefferson Medical College of Thomas Jefferson University

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

Disclosure: Nothing to disclose.

Additional Contributors

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.

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Factor XI deficiency. Graph depicts factor deficiencies.
 
 
 
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