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Pediatric Factor VII Deficiency Treatment & Management

  • Author: Helge Dirk Hartung, MD; Chief Editor: Max J Coppes, MD, PhD, MBA  more...
 
Updated: Oct 06, 2015
 

Medical Care

Acute bleeds: Management of acute hemorrhage primarily consists of factor VII (FVII) replacement therapy to treat bleeding. levels of more than 10% are usually hemostatic, although higher levels may be advisable in the event of a severe bleeding episode. Because factor VII has a short half-life (3-4 h), repeat treatment may be necessary in all except minor bleeding episodes. Treatment alternatives include the following:

  • Fresh frozen plasma is the least effective because of the volume required to provide adequate factor VII replacement. No viral attenuation of this product means that a risk of viral transmission is present.
  • Prothrombin complex concentrates contain factors II, IX, and X in addition to factor VII. These concentrates have undergone viral attenuation during manufacturing. Determining the appropriate dosage for treatment of factor VII deficiency can be difficult. These agents carry a risk of thrombogenic complications, particularly with repeated administration.
  • Factor VII concentrates are purified plasma–derived preparations that have undergone a vapor-heat viral-inactivation process. If available, factor VII concentrates are preferred over untreated plasma.[6] When given at high doses, these concentrates carry a risk of thrombosis, likely because of other vitamin K-dependent factors that are present in significant concentrations.
  • Recombinant activated factor VII (rFVIIa) was originally developed to treat patients with hemophilia and inhibitors, but it can be used at lower doses for patients with congenital factor VII deficiency. With increasing experience and evaluation of rFVIIa for treatment and prophylaxis in factor VII deficiency, the benefits and safety profile in this setting are becoming clearer. Arterial thromboembolic events, a concern in adult patients treated with high doses of rFVIIa,[7] have not been found at an increased rate.

Prophylaxis: The decision to embark on a program of prophylaxis is determined by the patient's clinical presentation and the number of clinically significant bleeding episodes requiring intervention. Consider prophylaxis for patients with recurrent hemarthrosis or intracranial hemorrhage. Beneficial results have been reported with regimens that vary from twice daily to twice weekly treatment.[8]

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Surgical Care

Maintaining factor VII levels of at least 15-25% provides adequate hemostasis levels for most surgical procedures.[9]

Preoperative factor VII replacement and monitoring of factor VII levels is essential for major surgical interventions.

Because of the short half-life (3-4 h), replacement therapy should continue postoperatively; the period of therapy is determined by the nature and extent of the procedure.

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Consultations

Consult a hematologist and/or hemostasis specialist for patients who require factor VII replacement therapy.

Genetic counseling and family studies are part of a complete evaluation.

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Activity

In patients with severe factor VII deficiency and a history of clinical bleeding, consider the risk of bleeding when choosing activities.

Individuals should stay fit because good muscle strength protects joints.

Patients are encouraged to avoid contact sports, wear appropriate protective gear, and choose activities, such as swimming, that promote muscle strength and flexibility with a low risk of joint injury.

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Contributor Information and Disclosures
Author

Helge Dirk Hartung, MD Attending Physician, Division of Hematology, The Children's Hospital of Philadelphia

Disclosure: Nothing to disclose.

Specialty Editor Board

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; Associate 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 Society of Pediatric Hematology/Oncology, American Federation for Clinical Research, Council on Medical Student Education in Pediatrics, Hemophilia and Thrombosis Research Society, American Academy of Pediatrics, American Association for Cancer Research, American Society of Hematology

Disclosure: Nothing to disclose.

Chief Editor

Max J Coppes, MD, PhD, MBA Executive Vice President, Chief Medical and Academic Officer, Renown Heath

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

Disclosure: Nothing to disclose.

Acknowledgements

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.

References
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  2. Acharya SS. Rare bleeding disorders in children: identification and primary care management. Pediatrics. 2013 Nov. 132 (5):882-92. [Medline].

  3. Tuddenham EG, Pemberton S, Cooper DN. Inherited factor VII deficiency: genetics and molecular pathology. Thromb Haemost. 1995 Jul. 74(1):313-21. [Medline].

  4. Mariani G, Herrmann FH, Dolce A, et al. Clinical phenotypes and factor VII genotype in congenital factor VII deficiency. Thromb Haemost. 2005 Mar. 93(3):481-7. [Medline].

  5. Peyvandi F, Mannucci PM. Rare coagulation disorders. Thromb Haemost. 1999 Oct. 82(4):1207-14. [Medline].

  6. Mariani G, Bernardi F. Factor VII Deficiency. Semin Thromb Hemost. 2009 Jun. 35(4):400-6. [Medline].

  7. Levi M, Levy JH, Andersen HF, Truloff D. Safety of recombinant activated factor VII in randomized clinical trials. N Engl J Med. 2010 Nov 4. 363(19):1791-800. [Medline].

  8. Todd T, J Perry D. A review of long-term prophylaxis in the rare inherited coagulation factor deficiencies. Haemophilia. 2009 Nov 11. [Medline].

  9. Kim SH, Park YS, Kwon KH, Lee JH, Kim KC, Yoo MC. Surgery in patients with congenital factor VII deficiency: A single center experience. Korean J Hematol. 2012 Dec. 47(4):281-5. [Medline]. [Full Text].

  10. Di Paola J, Nugent D, Young G. Current therapy for rare factor deficiencies. Haemophilia. 2001 Jan. 7 Suppl 1:16-22. [Medline].

  11. Giansily-Blaizot M, Schved JF. Potential predictors of bleeding risk in inherited factorVII deficiency. Clinical, biological and molecular criteria. Thromb Haemost. 2005 Nov. 94(5):901-6. [Medline].

  12. Mariani G, Dolce A, Marchetti G, Bernardi F. Clinical picture and management of congenital factor VII deficiency. Haemophilia. 2004 Oct. 10 Suppl 4:180-3. [Medline].

  13. Mariani G, Lapecorella M, Dolce A. Steps towards an effective treatment strategy in congenital factor VII deficiency. Semin Hematol. 2006 Jan. 43(1 Suppl 1):S42-7. [Medline].

  14. Perry DJ. Factor VII Deficiency. Br J Haematol. 2002 Sep. 118(3):689-700. [Medline].

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Intrinsic and extrinsic pathways of coagulation. Factor VII/tissue factor complex activates factor IX and factor X. Factor IXa along with factor VIIIa results in formation of more factor Xa. Factor Xa along with factor Va converts prothrombin to thrombin.
 
 
 
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