Inherited Abnormalities of Fibrinogen Treatment & Management

Author: Vinod V Balasa, MD; Chief Editor: Max J Coppes, MD, PhD, MBA more...

Updated: Jul 28, 2010

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Medical Care
Surgical Care
Consultations
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Medical Care

Hemorrhage

For patients with clinical bleeding associated with afibrinogenemia or dysfibrinogenemia, replacement of fibrinogen to a level of more than 0.8 g/L is usually adequate to maintain hemostasis, although levels greater than 1 g/L have been recommended for CNS hemorrhage. Plasma-derived fibrinogen concentrates have the advantage of virus inactivation. The usual starting dose for adults is 1-2 g intravenously administered. The pediatric dose is 30-100 mg/kg intravenously administered, depending on the severity and site of bleeding. Cryoprecipitate has been used as a source of fibrinogen; each bag of cryoprecipitate contains 100-250 mg of fibrinogen. The guidelines for dysfibrinogenemia are not standardized due to a lack of sufficient data in bleed management.

Fibrinogen dosage calculation ^[7]

Dose (g) = desired increment in g/L x plasma volume (plasma volume is 0.07 x (1-hematocrit) x weight (kg)

The patient's personal and family history of bleeding and thrombosis should be taken into consideration for appropriate dosing of replacement therapy. In addition, the pharmacokinetics of fibrinogen after replacement therapy widely varies, and individual dose adjustment is recommended.

Thrombosis

Patients who present with thrombosis associated with dysfibrinogenemia should receive anticoagulation therapy. The duration of therapy has not been established for this particular group of patients; the decision depends on the clinical situation and the presence of other contributing factors. If the patient has had multiple thromboembolic events, a single life-threatening event, or has additional inherited risk factors, protracted anticoagulation therapy is recommended.

Spontaneous abortion

Recurrent spontaneous abortion may be prevented by routine prophylaxis with fibrinogen concentrates starting early in pregnancy.

Acquired inhibitors

Acquired inhibitors have been reported after replacement therapy and should be considered in previously treated patients who demonstrate poor hemostasis with usual therapies.

Surgical Care

To prevent excessive bleeding during surgical procedures, prophylactic treatment to raise fibrinogen levels to 1-1.5 g/L during the procedure is recommended. Replacement should be continued for 4-14 days following surgery, depending on the nature of the surgical procedure and time to complete healing.

Consultations

Consultation with a hematologist/hemostasis specialist is advisable for patients who require fibrinogen replacement therapy. Genetic counseling and family studies should be part of a complete evaluation.

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

Helen SL 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

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The conversion of soluble fibrinogen to insoluble fibrin.

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