Diagnostic Considerations

Acquired factor VII (FVII) deficiency due to vitamin K deficiency, vitamin K antagonist therapy, or liver disease: In these situations, reduced factor VII levels are associated with reduced levels of other vitamin K–dependent factors. Acquired factor VII deficiency is far more common than inherited deficiency.

Other congenital coagulation factor deficiencies

Workup

References

Mandhyan R, Tiwari A, Cherian G. Congenital factor VII deficiency. Br J Anaesth. 2010 Feb. 104(2):267-8. [QxMD MEDLINE Link].
Acharya SS. Rare bleeding disorders in children: identification and primary care management. Pediatrics. 2013 Nov. 132 (5):882-92. [QxMD MEDLINE Link].
Tuddenham EG, Pemberton S, Cooper DN. Inherited factor VII deficiency: genetics and molecular pathology. Thromb Haemost. 1995 Jul. 74(1):313-21. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Jain S, Donkin J, Frey MJ, Peltier S, Gunawardena S, Cooper DL. Phenotypical variability in congenital FVII deficiency follows the ISTH-SSC severity classification guidelines: a review with illustrative examples from the clinic. J Blood Med. 2018. 9:211-18. [QxMD MEDLINE Link]. [Full Text].
Peyvandi F, Mannucci PM. Rare coagulation disorders. Thromb Haemost. 1999 Oct. 82(4):1207-14. [QxMD MEDLINE Link].
Mariani G, Bernardi F. Factor VII Deficiency. Semin Thromb Hemost. 2009 Jun. 35(4):400-6. [QxMD MEDLINE Link].
Neufeld EJ, Negrier C, Arkhammar P, et al. Safety update on the use of recombinant activated factor VII in approved indications. Blood Rev. 2015 Jun. 29 Suppl 1:S34-41. [QxMD MEDLINE Link].
Ramezanpour N, Zaker F, Biswas A, Dorgalaleh A. Inhibitor in Congenital Factor VII Deficiency; a Rare but Serious Therapeutic Challenge-A Systematic Literature Review. J Clin Med. 2021 Jan 8. 10 (2):[QxMD MEDLINE Link]. [Full Text].
Todd T, J Perry D. A review of long-term prophylaxis in the rare inherited coagulation factor deficiencies. Haemophilia. 2009 Nov 11. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link]. [Full Text].
Rajpurkar M, Cooper DL. Continuous infusion of recombinant activated factor VII: a review of data in congenital hemophilia with inhibitors and congenital factor VII deficiency. J Blood Med. 2018. 9:227-39. [QxMD MEDLINE Link]. [Full Text].

Media Gallery

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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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

Hassan M Yaish, MD Medical Director, Intermountain Hemophilia and Thrombophilia Treatment Center; Professor of Pediatrics, University of Utah School of Medicine; Director of Hematology, Pediatric Hematologist/Oncologist, Department of Pediatrics, Primary Children's Medical Center

Hassan M Yaish, MD is a member of the following medical societies: American Academy of Pediatrics, American Medical Association, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Michigan State Medical Society, New York Academy of Sciences

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.