Factor VII Deficiency Workup

1. Ferraresi P, Balestra D, Guittard C, Buthiau D, Pan-Petesh B, Maestri I, et al. Next-generation sequencing and recombinant expression characterized aberrant splicing mechanisms and provided correction strategies in factor VII deficiency. Haematologica. 2019 Jul 4. [Medline]. [Full Text].

2. Napolitano M, Siragusa S, Mariani G. Factor VII Deficiency: Clinical Phenotype, Genotype and Therapy. J Clin Med. 2017 Mar 28. 6 (4):[Medline]. [Full Text].

3. Alexander B, R Goldstein and G Landwehr, CooK CD. Congenital SPCA deficiency: a hitherto unrecognized coagulation defect with hemorrhage rectified by serum and serum fractions. J Clin Invest. 1951 Jun. 30(6):596-608. [Medline].

4. Roberts HR, Monroe DM, Hoffman M. Molecular biology and biochemistry of the coagulation factors and pathways of hemostasis. Beutler E, Lichtman MA, Coller BS, Kipps TJ, Seligsohn U, eds. William's Hematology. 6th ed. New York, NY: McGraw-Hill; 2001. 2001: 1409-34.

5. Lane A, Cruickshank JK, Mitchell J, et al. Genetic and environmental determinants of factor VII coagulant activity in ethnic groups at differing risk of coronary heart disease. Atherosclerosis. 1992 May. 94(1):43-50. [Medline].

6. Pinotti M, Toso R, Girelli D, et al. Modulation of factor VII levels by intron 7 polymorphisms: population and in vitro studies. Blood. 2000 Jun 1. 95(11):3423-8. [Medline].

7. Laurian Y. Treatment of bleeding in patients with platelet disorders: is there a place for recombinant factor VIIa?. Pathophysiol Haemost Thromb. 2002. 32 Suppl 1:37-40. [Medline].

8. Geng Y, Verhamme IM, Sun MF, Paul Bajaj S, Emsley J, Gailani D. Analysis of the factor XI variant Arg184Gly suggests a structural basis for factor IX binding to factor XIa. J Thromb Haemost. 2013 Apr 26. [Medline].

9. Herrmann FH, Wulff K, Auberger K, et al. Molecular biology and clinical manifestation of hereditary factor VIIdeficiency. Semin Thromb Hemost. 2000. 26(4):393-400. [Medline].

10. Bernardi F, Patracchini P, Gemmati D, et al. Molecular analysis of factor VII deficiency in Italy: a frequent mutation(FVII Lazio) in a repeated intronic region. Hum Genet. 1993 Nov. 92(5):446-50. [Medline].

11. Cutler JA, Patel R, Mitchell MJ, Savidge GF. The significance of published polymorphisms in 14 cases of mild factor VII deficiency. Blood Coagul Fibrinolysis. 2005 Mar. 16(2):91-5. [Medline].

12. Meade TW, Ruddock V, Stirling Y, et al. Fibrinolytic activity, clotting factors, and long-term incidence ofischaemic heart disease in the Northwick Park Heart Study. Lancet. 1993 Oct 30. 342(8879):1076-9. [Medline].

13. Moor E, Silveira A, van't Hooft F, Suontaka AM, Eriksson P, Blombäck M, et al. Coagulation factor VII mass and activity in young men with myocardial infarction at a young age. Role of plasma lipoproteins and factor VII genotype. Arterioscler Thromb Vasc Biol. 1995 May. 15(5):655-64. [Medline].

14. Rodgers GM, Greenberg CS. Inherited coagulation disorders. Lee GR, Foerster J, Lukens J, Paraskevas F, Greer JP, Rodgers GM, eds. Wintrobe's Clinical Hematology. 10th ed. Baltimore, Md: Williams & Wilkins; 1999. Vol 2: 1682-732.

15. Heywood DM, Carter AM, Catto AJ, et al. Polymorphisms of the factor VII gene and circulating FVII:C levels in relation to acute cerebrovascular disease and poststroke mortality. Stroke. 1997 Apr. 28(4):816-21. [Medline].

16. Friederich PW, Henny CP, Messelink EJ, et al. Effect of recombinant activated factor VII on perioperative blood loss in patients undergoing retropubic prostatectomy: a double-blind placebo-controlled randomised trial. Lancet. 2003 Jan 18. 361(9353):201-5. [Medline].

17. Blechman S, Fruchtman Y, Perry ZH, Mazar J, Ben Harosh M, Abed A, et al. Clinical and Laboratory Findings in Jewish and Bedouin Patients in Southern Israel Who Were Diagnosed with Factor VII Deficiency. Isr Med Assoc J. 2019 May. 5 (21):318-321. [Medline]. [Full Text].

18. Seligsohn U, White GC. Inherited deficiencies of coagulation factors II, V, VII, XI and XIII and the combined deficiencies of factors V and VII and of the vitamin K-dependent factors. Beutler E, Lichtman MA, Coller BS, Kipps TJ, Seligsohn U, eds. William's Hematology. 6th ed. New York, NY: McGraw-Hill; 2001. 1617-38.

19. 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].

20. Zantek ND, Hsu P, Refaai MA, Ledford-Kraemer M, Meijer P, Van Cott EM. Factor VII assay performance: an analysis of the North American Specialized Coagulation Laboratory Association proficiency testing results. Int J Lab Hematol. 2013 Jun. 35(3):314-21. [Medline].

21. 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].

22. Napolitano M, Di Minno MN, Batorova A, Dolce A, Giansily-Blaizot M, Ingerslev J, et al. Women with congenital factor VII deficiency: clinical phenotype and treatment options from two international studies. Haemophilia. 2016 Sep. 22 (5):752-9. [Medline].

23. Di Minno MND, Napolitano M, Dolce A, Mariani G, STER Study Group. Role of clinical and laboratory parameters for treatment choice in patients with inherited FVII deficiency undergoing surgical procedures: evidence from the STER registry. Br J Haematol. 2018 Feb. 180 (4):563-570. [Medline]. [Full Text].

24. 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. 9:227-239. [Medline]. [Full Text].

25. Siboni SM, Biguzzi E, Mistretta C, Garagiola I, Peyvandi F. Long-term prophylaxis in severe factor VII deficiency. Haemophilia. 2015 Nov. 21 (6):812-9. [Medline].

26. Kuperman AA, Barg AA, Fruchtman Y, Shaoul E, Rosenberg N, Kenet G, et al. Primary prophylaxis for children with severe congenital factor VII deficiency - Clinical and laboratory assessment. Blood Cells Mol Dis. 2017 Sep. 67:86-90. [Medline].

27. Gruppo RA, Kessler CM, Neufeld EJ, Cooper DL. Assessment of individual dose utilization vs. physician prescribing recommendations for recombinant activated factor VII (rFVIIa) in paediatric and adult patients with congenital haemophilia and alloantibody inhibitors (CHwI): the Dosing Observational Study in Hemophilia (DOSE). Haemophilia. 2013 Apr 1. [Medline].

28. Ludlam CA. The evidence behind inhibitor treatment with recombinant factor VIIa. Pathophysiol Haemost Thromb. 2002. 32 Suppl 1:13-8. [Medline].

29. Lisman T, Moschatsis S, Adelmeijer J, et al. Recombinant factor VIIa enhances deposition of platelets with congenital or acquired alpha IIb beta 3 deficiency to endothelial cell matrix and collagen under conditions of flow via tissue factor-independent thrombin generation. Blood. 2003 Mar 1. 101(5):1864-70. [Medline].

30. Ludlam CA, Smith MP, Morfini M, et al. A prospective study of recombinant activated factor VII administered by continuous infusion to inhibitor patients undergoing elective major orthopaedic surgery: a pharmacokinetic and efficacy evaluation. Br J Haematol. 2003 Mar. 120(5):808-13. [Medline].

31. Logan AC, Yank V, Stafford RS. Off-Label Use of Recombinant Factor VIIa in U.S. Hospitals: Analysis of Hospital Records. Ann Intern Med. 2011 Apr 19. 154(8):516-22. [Medline].

32. Logan AC, Goodnough LT. Recombinant factor VIIa: an assessment of evidence regarding its efficacy and safety in the off-label setting. Hematology Am Soc Hematol Educ Program. 2010. 2010:153-9. [Medline].

33. Butenas S, Brummel KE, Branda RF, et al. Mechanism of factor VIIa-dependent coagulation in hemophilia blood. Blood. 2002 Feb 1. 99(3):923-30. [Medline].

34. Monroe DM, Hoffman M, Oliver JA, Roberts HR. A possible mechanism of action of activated factor VII independent of tissue factor. Blood Coagul Fibrinolysis. 1998 Mar. 9 Suppl 1:S15-20. [Medline].

35. Mayer SA, Brun NC, Begtrup K, et al. Recombinant activated factor VII for acute intracerebral hemorrhage. N Engl J Med. 2005 Feb 24. 352(8):777-85. [Medline].

36. Barletta JF, Ahrens CL, Tyburski JG, Wilson RF. A review of recombinant factor VII for refractory bleeding in nonhemophilic trauma patients. J Trauma. 2005 Mar. 58(3):646-51. [Medline].

Muhammad A Mir, MD, FACP Assistant Professor of Medicine (Hematology, Blood/Marrow Transplant) Milton S Hershey Medical Center, Pennsylvania State University College of Medicine

Muhammad A Mir, MD, FACP is a member of the following medical societies: American College of Physicians, American Society of Hematology, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology

Disclosure: Nothing to disclose.

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, MBBCh, FRCPC, DTM&H Professor of Internal Medicine and Pathology, Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center

Ronald A Sacher, MBBCh, 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.

Perumal Thiagarajan, MD Professor, Department of Pathology and Medicine, Baylor College of Medicine; Director, Transfusion Medicine and Hematology Laboratory, Michael E DeBakey Veterans Affairs Medical Center

Perumal Thiagarajan, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Society for Biochemistry and Molecular Biology, American Society for Clinical Investigation, American Society of Hematology, Association of American Physicians, Royal College of Physicians

Disclosure: Nothing to disclose.

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.

Francisco J Hernandez-Ilizaliturri, MD Associate Professor of Medicine, Department of Medicine, Assistant Professor of Immunology, Department of Immunology, Roswell Park Cancer Institute, University of Buffalo State University of New York School of Medicine and Biomedical Sciences

Francisco J Hernandez-Ilizaliturri, MD is a member of the following medical societies: American Association for Cancer Research and American Society of Hematology

Disclosure: Nothing to disclose.

Ganapathy S Krishnan, MBBS Fellow, Department of Hematology and Oncology, Michigan State University

Ganapathy S Krishnan, MBBS is a member of the following medical societies: American Society of Hematology

Disclosure: Nothing to disclose.

Jeyanthi Ramanarayanan, MD Assistant Professor, Medical Oncology, Veterans Affairs Medical Center of Buffalo

Jeyanthi Ramanarayanan, MD is a member of the following medical societies: American Association of Physicians of Indian Origin and American Society of Hematology

Disclosure: Nothing to disclose.

• Print this section
• Print the entire contents of
• Print the entire contents of article