eMedicine Specialties > Hematology > Coagulation, Hemostasis, and Disorders

Factor V: Differential Diagnoses & Workup

Author: Olga Kozyreva, MD, Fellow, Department of Hematology-Oncology, Tufts Medical Center, Tufts University School of Medicine
Coauthor(s): Sarah K May, MD, Consulting Staff, Department of Hematology-Oncology, Caritas Carney Hospital, Commonwealth Hematology-Oncology PC; Karl J D'Silva, MD, Fellow in Hematology/Oncology, Department of Internal Medicine, Breslin Cancer Center, Michigan State University
Contributor Information and Disclosures

Updated: Aug 29, 2009

Differential Diagnoses

Platelet Disorders
von Willebrand Disease

Other Problems to Be Considered

All other coagulation disorders

Workup

Laboratory Studies

  • The presence of a mild prolongation of PT and aPTT may be the first indication of factor V deficiency. Use specific factor V activity and antigen assays for confirmation.
    • Bleeding time can be prolonged in severe cases.
      • aPTT - Prolonged
      • PT - Prolonged
      • Thrombin time - Normal
      • Stypven time (Russell viper venom time [RVVT]) - Prolonged
    • Mixing study - Correction of PT or partial thromboplastin time (PTT) with the mixing of equal amounts of normal and patient plasma
    • Factor V inhibitor panel
    • Factor V antigen - Quantifies factor V levels but does not test for functional factor V. Some patients have factor V deficiency caused by dysfunctional factor V while the level of factor V protein, estimated by factor V antigen levels, is normal. However, this test is not routinely ordered to diagnose factor V deficiency.

Imaging Studies

  • Early and aggressive imaging studies are indicated, even with low suspicion for hemorrhage, after coagulation therapy is initiated.
  • Head CT scan (noncontrast): Assess spontaneous or traumatic hemorrhage.
  • Body CT scan

    • Perform with or without intravenous contrast, oral contrast, or both.
    • Perform as indicated by clinical suspicion and anatomical location.
    • Assess spontaneous or traumatic hemorrhage.
  • Head and spinal column MRI: Further assess spontaneous or traumatic hemorrhage.
  • Radiograph for joint assessment: This is of limited value in an acute setting of hemarthrosis. Chronic degenerative joint disease is often present.
  • Special studies: Perform angiography and nucleotide bleeding scan as clinically indicated.

More on Factor V

Overview: Factor V
Differential Diagnoses & Workup: Factor V
Treatment & Medication: Factor V
Follow-up: Factor V
Multimedia: Factor V
References
Further Reading
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References

  1. Owren P. Parahaemophilia: haemorrhagic diathesis due to absence of a previously unknown clotting factor. Lancet. 1947;Vol I:446-8.

  2. Duckers C, Simioni P, Rosing J, Castoldi E. Advances in understanding the bleeding diathesis in factor V deficiency. Br J Haematol. Jun 2009;146(1):17-26. [Medline].

  3. Asselta R, Peyvandi F. Factor V deficiency. Semin Thromb Hemost. Jun 2009;35(4):382-9. [Medline].

  4. Ni Ainle F, Preston RJ, Jenkins PV, et al. Protamine sulfate down-regulates thrombin generation by inhibiting factor V activation. Blood. Aug 20 2009;114(8):1658-65. [Medline].

  5. Delev D, Pavlova A, Heinz S, Seifried E, Oldenburg J. Factor 5 mutation profile in German patients with homozygous and heterozygous factor V deficiency. Haemophilia. Sep 2009;15(5):1143-53. [Medline].

  6. Fu YX, Kaufman R, Rudolph AE, Collum SE, Blinder MA. Multimodality therapy of an acquired factor V inhibitor. Am J Hematol. Apr 1996;51(4):315-8. [Medline].

  7. Spreafico M, Peyvandi F. Combined factor V and factor VIII deficiency. Semin Thromb Hemost. Jun 2009;35(4):390-9. [Medline].

  8. Esmon CT. Basic mechanisms and pathogenesis of venous thrombosis. Blood Rev. Sep 2009;23(5):225-9. [Medline].

  9. Girolami A, Scandellari R, Lombardi AM, Girolami B, Bortoletto E, Zanon E. Pregnancy and oral contraceptives in factor V deficiency: a study of 22 patients (five homozygotes and 17 heterozygotes) and review of the literature. Haemophilia. Jan 2005;11(1):26-30. [Medline].

  10. Ichikawa H. Successful total gastrectomy of gastric cancer in a congenital factor V deficient patient [in Japanese]. Nippon Ronen Igakkai Zasshi. Mar 2000;37(3):245-9. [Medline].

  11. Lak M, Sharifian R, Peyvandi F, Mannucci PM. Symptoms of inherited factor V deficiency in 35 Iranian patients. Br J Haematol. Dec 1998;103(4):1067-9. [Medline].

  12. Lee GR, Foerster J, Greer J, et al, eds. Wintrobe's Clinical Hematology. Vol 2. 10th ed. Baltimore, Md: Lippincott, Williams & Wilkins; 1999:1710-12.

  13. Mann KG, Kalafatis M. Factor V: a combination of Dr Jekyll and Mr Hyde. Blood. Jan 1 2003;101(1):20-30. [Medline].

  14. Mitterstieler G, Muller W, Geir W. Congenital factor V deficiency. A family study. Scand J Haematol. Jul 1978;21(1):9-13. [Medline].

  15. Nowak-Gottl U, Weiler H, Hernandez I, et al. Fibrinogen alpha and gamma genes and factor VLeiden in children with thromboembolism: results from 2 family-based association studies. Blood. Aug 27 2009;114(9):1947-53. [Medline].

  16. Sacco S, Dragani A, Davi G, Carolei A. Four recurrent intracerebral haemorrhages. Cerebrovasc Dis. 2003;16(4):435-6. [Medline].

  17. Satoh H, Yamashita YT, Ohtsuka M, Sekizawa K, Hasegawa Y. Pulmonary hemorrhage in factor V deficiency. Can Respir J. Jul-Aug 1999;6(4):320. [Medline].

  18. Song JW, Um MR, Ahn HS, Hong CY. A case of congenital factor V deficiency. J Korean Med Sci. Sep 1987;2(3):179-82. [Medline].

  19. Suehisa E, Kawasaki T, Toku M, Hidaka Y. Low level of factor V is associated with development of deep-vein thrombosis in Japanese patients. Thromb Res. Jun 2 2009;[Medline].

  20. Totan M, Albayrak D. Intracranial haemorrhage due to factor V deficiency. Acta Paediatr. Mar 1999;88(3):342-3. [Medline].

  21. Tracy PB, Mann KG. Abnormal formation of the prothrombinase complex: factor V deficiency and related disorders. Hum Pathol. Feb 1987;18(2):162-9. [Medline].

  22. Yokoyama T, Tatemoto Y, Osaki T. Hemostatic treatment after tooth extraction in a patient with factor V deficiency. Oral Dis. Sep 1997;3(3):196-8. [Medline].

  23. Yotsumoto G, Masuda H, Toyokawa K, Iguro Y, Kinjo T, Sakata R. Off-pump coronary artery bypass grafting in a patient with congenital factor V deficiency: report of a case. Surg Today. 2005;35(2):142-4. [Medline].

  24. Zehnder JL, Hiraki DD, Jones CD, Gross N, Grumet FC. Familial coagulation factor V deficiency caused by a novel 4 base pair insertion in the factor V gene: factor V Stanford. Thromb Haemost. Sep 1999;82(3):1097-9. [Medline].

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Keywords

factor V, FV, factor V deficiency, hereditary factor V deficiency disease, proaccelerin deficiency, accelerator globulin deficiency, AC globulin deficiency, hereditary hypoproaccelerinemia, hemophilia, parahemophilia, anticoagulation, coagulopathy anticoagulant, coagulation factors, hemostatic pathway, haemostatic pathway, coagulation pathway, acquired factor V inhibitor, coagulation factor V,
 
Thrombosis, deep venous thrombosis, thrombin, prothrombinase complex, platelet activation, platelet factor V, prothrombinase function, prothrombin time, PT, activated partial thromboplastin time, aPTT, partial thromboplastin time, PTT, factor IX, FIX, factor VIII, FVIII, bleeding disorder, labile factor deficiency, von Willebrand factor, von Willebrand's factor, vWF, von Willebrand disease, von Willebrand's disease, vWD, Owren disease, Owren's disease

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Contributor Information and Disclosures

Author

Olga Kozyreva, MD, Fellow, Department of Hematology-Oncology, Tufts Medical Center, Tufts University School of Medicine
Disclosure: Nothing to disclose.

Coauthor(s)

Sarah K May, MD, Consulting Staff, Department of Hematology-Oncology, Caritas Carney Hospital, Commonwealth Hematology-Oncology PC
Disclosure: Nothing to disclose.

Karl J D'Silva, MD, Fellow in Hematology/Oncology, Department of Internal Medicine, Breslin Cancer Center, Michigan State University
Karl J D'Silva, MD is a member of the following medical societies: Massachusetts Medical Society
Disclosure: Nothing to disclose.

Medical Editor

Paul Schick, MD, Emeritus Professor, Department of Internal Medicine, Thomas Jefferson University Medical College; Research Professor, Department of Internal Medicine, Drexel University College of Medicine; Adjunct Professor of Medicine, Lankenau Hospital, Wynnewood, PA
Paul Schick, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Society of Hematology, International Society on Thrombosis and Haemostasis, and New York Academy of Sciences
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Ronald A Sacher, MB, BCh, MD, FRCPC, Professor, Internal Medicine and Pathology, Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center
Ronald A Sacher, MB, BCh, MD, FRCPC is a member of the following medical societies: American Society of Hematology
Disclosure: Glaxo Smith Kline Honoraria Speaking and teaching; Talecris Honoraria Board membership

CME Editor

Rebecca J Schmidt, DO, FACP, FASN, Professor of Medicine, Section Chief, Department of Medicine, Section of Nephrology, West Virginia University School of Medicine
Rebecca J Schmidt, DO, FACP, FASN is a member of the following medical societies: American College of Osteopathic Internists, American College of Physicians, American Medical Association, American Society of Nephrology, International Society of Nephrology, National Kidney Foundation, Renal Physicians Association, and West Virginia State Medical Association
Disclosure: Abbott Grant/research funds Speaking and teaching; Genzyme Honoraria Consulting; Amgen Honoraria Speaking and teaching; Ortho Biotech Honoraria Speaking and teaching

Chief Editor

Emmanuel C Besa, MD, Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Thomas Jefferson University
Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, and New York Academy of Sciences
Disclosure: Nothing to disclose.

 
 
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