Inherited Abnormalities of Fibrinogen Medication

  • Author: Vinod V Balasa, MD; Chief Editor: Max J Coppes, MD, PhD, MBA   more...
 
Updated: May 16, 2012
 

Plasma sources of fibrinogen

Class Summary

Fibrinogen concentrates and cryoprecipitate are both derived from human plasma. The advantage of fibrinogen concentrates is that virus inactivation is incorporated into the preparation of this product. More precise dosing can be achieved with concentrates, as the fibrinogen concentration in cryoprecipitate may vary.

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Fibrinogen concentrate, human (RiaSTAP)

 

Approved by the FDA in 2009. Indicated for the treatment of acute bleeding episodes in patients with congenital fibrinogen deficiency, including afibrinogenemia and hypofibrinogenemia. Not indicated for dysfibrinogenemia. Available as single-use vials containing 900-1300 mg lyophilized fibrinogen concentrate powder for reconstitution. Actual fibrinogen potency for each lot is printed on vial label and carton.

Fibrinogen concentrate (Haemocomplettan P, Clottagen, Fibrinogen HT)

 

Fibrinogen concentrates are derived from human plasma and have been virus-inactivated to improve their safety. In the United States, human fibrinogen is available as an orphan drug from Alpha Therapeutics.

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Cryoprecipitate

 

Can be used when fibrinogen concentrates are not available. Unlike fibrinogen concentrates, it does not undergo virus inactivation. The precipitate formed when fresh frozen plasma (FFP) is slowly thawed. It contains factor VIII, factor XIII, fibrinogen, von Willebrand factor (vWF), and fibronectin. Each bag provides 100-250 mg fibrinogen.

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Antifibrinolytics

Class Summary

These are useful in conjunction with fibrinogen replacement for the treatment of mucosal bleeding, particularly bleeding involving the oronasopharynx. Inhibition of local fibrinolysis allows maintenance of the clot and decreases the frequency of rebleeding.

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Aminocaproic acid (Amicar)

 

Lysine analogue that inhibits fibrinolysis by blocking binding of plasmin or plasminogen activators to lysine residues on fibrin.

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Tranexamic acid (Cyklokapron)

 

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

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Contributor Information and Disclosures
Author

Vinod V Balasa, MD  Associate Professor of Pediatrics, Director of Hemophilia and Thrombosis Clinic, Division of Pediatric Hematology and Oncology, Department of Pediatrics, University of Louisville School of Medicine

Vinod V Balasa, MD is a member of the following medical societies: American Society of Hematology, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, Hemophilia and Thrombosis Research Society, Indian Medical Association, and International Society on Thrombosis and Haemostasis

Disclosure: Nothing to disclose.

Specialty Editor Board

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.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

James L Harper, MD  Associate Professor, Department of Pediatrics, Division of Hematology/Oncology and Bone Marrow Transplantation, Associate Chairman for Education, Department of Pediatrics, University of Nebraska Medical Center; Assistant Clinical Professor, Department of Pediatrics, Creighton University School of Medicine; Director, Continuing Medical Education, Children's Memorial Hospital; Pediatric Director, Nebraska Regional Hemophilia Treatment Center

James L Harper, MD is a member of the following medical societies: American Academy of Pediatrics, American Association for Cancer Research, American Federation for Clinical Research, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Council on Medical Student Education in Pediatrics, and Hemophilia and Thrombosis Research Society

Disclosure: Nothing to disclose.

Helen SI Chan, MBBS, FRCP(C), FAAP  Associate Senior Scientist, Research Institute; Professor, Division of Hematology/Oncology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto Faculty of Medicine, Canada

Helen SI Chan, MBBS, FRCP(C), FAAP is a member of the following medical societies: American Academy of Pediatrics, American Association for Cancer Research, American Society of Hematology, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Chief Editor

Max J Coppes, MD, PhD, MBA  Senior Vice President, Center for Cancer and Blood Disorders, Children's National Medical Center; Professor of Medicine, Oncology, and Pediatrics, Georgetown University School of Medicine; Clinical Professor of Pediatrics, George Washington University School of Medicine and Health Sciences

Max J Coppes, MD, PhD, MBA is a member of the following medical societies: American Association for Cancer Research, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

References

  1. Bolton-Maggs PH, Perry DJ, Chalmers EA, et al. 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].

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  5. Haverkate F, Samama M. Familial dysfibrinogenemia and thrombophilia. Report on a study of the SSC Subcommittee on Fibrinogen. Thromb Haemost. Jan 1995;73(1):151-61. [Medline].

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  7. Acharya SS, Dimichele DM. Rare inherited disorders of fibrinogen. Haemophilia. Nov 2008;14(6):1151-8. [Medline].

  8. Parameswaran R, Dickinson JP, de Lord S, et al. Spontaneous intracranial bleeding in two patients with congenital afibrinogenaemia and the role of replacement therapy. Haemophilia. Nov 2000;6(6):705-8. [Medline].

  9. Martinez J. Congenital dysfibrinogenemia. Curr Opin Hematol. Sep 1997;4(5):357-65. [Medline].

  10. Verhovsek M, Moffat KA, Hayward CP. Laboratory testing for fibrinogen abnormalities. Am J Hematol. Dec 2008;83(12):928-31. [Medline].

  11. Cunningham MT, Brandt JT, Laposata M, Olson JD. Laboratory diagnosis of dysfibrinogenemia. Arch Pathol Lab Med. 2002;126:499-505. [Medline].

  12. Peyvandi F, Cattaneo M, Inbal A, De Moerloose P, Spreafico M. Rare bleeding disorders. Haemophilia. Jul 2008;14 Suppl 3:202-10. [Medline].

 
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The conversion of soluble fibrinogen to insoluble fibrin.
 
 
 
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