eMedicine Specialties > Hematology > Coagulation, Hemostasis, and Disorders

Factor V: Treatment & Medication

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

Treatment

Medical Care

No concentrates of factor V are commercially available, so fresh plasma or fresh frozen plasma (FFP) infusions are used to correct the deficiency temporarily and should be given daily during a bleeding episode. The loading dose of FFP is 15-20 mL/kg and then 3-6 mL/kg daily. Subsequent dosages depend on monitoring the factor V level by obtaining peak and trough factor V level assays. The half-life ranges from 24-36 hours, with the aim being a factor V level of 25%. Fluid overload and viral transmission may be a complication of plasma therapy.

  • Preoperative and postoperative care when patients with factor V deficiency require surgery include the following:
    • In pregnant women, considerable bleeding can occur at the time of delivery; however, the administration of FFP can properly control bleeding, with excellent fetal outcome.
    • The safe level of factor V for adequate surgical hemostasis is 25% of the activity of factor V in normal control plasma.
    • Postoperatively, FFP should be administered for 3-10 days, with careful observation of wound bleeding.
    • Tooth extraction in a patient with factor V hereditary deficiency is safely performed with both supplementation of FFP and application of local hemostasis.
  • Patients with factor V deficiency can be given oral contraceptives to decrease menometrorrhagias, thereby improving anemia and decreasing transfusion needs.
  • The optimal treatment of patients with factor V inhibitors is uncertain. Fu et al were successful in using a combination of factor replacement, chemotherapy, and plasmapheresis in a patient with spontaneous, life-threatening intracranial bleeding caused by a factor V inhibitor. The patient deteriorated after initial treatment with FFP and platelet transfusions. He was subsequently treated with a combination of plasma exchange and chemotherapy, and he completely recovered.6 The experience of Fu et al shows that combinations of therapies may be needed in patients with serious hemorrhage caused by acquired factor V deficiency.

Consultations

Consultations may include hematologists, blood bank specialists, pathologists, and others as indicated based on hemorrhagic complications.

Medication

The goals of pharmacotherapy are to reduce morbidity and to prevent complications.

Blood products

Plasma is the fluid compartment of blood that contains the soluble clotting factors.


Fresh frozen plasma

For use in patients with blood product deficiencies.

Adult

Dose depends on severity of coagulopathy
Initially, 2 U are administered IV , then more is administered as needed to control bleeding; monitor at peak and trough using a factor V assay to fine-tune the administration of FFP

Pediatric

Administer as in adults; administer 2 U IV initially; further administration depends on severity of coagulopathy

Pregnancy

A - Fetal risk not revealed in controlled studies in humans

Precautions

Viral contamination and infection are possible but unlikely because of prescreening; ineffective in patients with factor V inhibitors; may induce an anamnestic response; fluid overload can occur with multiple infusion and should be borne in mind

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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