Medication Summary

Medications for prophylaxis and treatment of bleeding in patients with factor XI deficiency include fresh frozen plasma and antifibrinolytic agents. Treatment choices are guided by patient factors and clinical circumstances.

Class Summary

To achieve a FXI level of 50%, a patient needs to have half of their plasma volume replaced.

Directed-donor fresh frozen plasma can be arranged for elective procedures, but a large volume of plasma will need to be stored to transfuse for the required 7-14 days after surgery. One study has demonstrated that solvent detergent fresh frozen plasma has a half-life of 45 hours, while a second study has shown that FXI is decreased in the product.

Fresh frozen plasma (FFP, Octaplas)

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Plasma is the fluid compartment of blood containing the soluble clotting factors. Octaplas is a solvent detergent treated, pooled FFP.

Class Summary

May consider prophylactic administration with antifibrinolytics prior to minor procedures (eg, dental).

Aminocaproic acid (Amicar)

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Inhibits fibrinolysis via inhibition of plasminogen activator substances and, to a lesser degree, through antiplasmin activity. Main problem is that the thrombi that form during treatment are not lysed, and effectiveness is uncertain.

Tranexamic acid injection (Cyklokapron)

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Alternative to aminocaproic acid. Inhibits fibrinolysis by displacing plasminogen from fibrin.

Questions

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Media Gallery

Factor XI deficiency. Diagram from the traditional cascade-waterfall model of coagulation shows the place of factor XI in the intrinsic pathway, which leads to the common pathway.

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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, MD, FRCPC, DTM&H Professor Emeritus of Internal Medicine and Hematology/Oncology, Emeritus Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center

Ronald A Sacher, MD, 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.

Chief Editor

Srikanth Nagalla, MD, MS, FACP Chief of Benign Hematology, Miami Cancer Institute, Baptist Health South Florida; Clinical Professor of Medicine, Florida International University, Herbert Wertheim College of Medicine

Srikanth Nagalla, MD, 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; Dova; Apellis; Pharmacosmos<br/>Serve(d) as a speaker or a member of a speakers bureau for: Sobi; Sanofi; Rigel.

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.