Further Outpatient Care

Ideally, a hematologist who has experience in the diagnosis and management of inherited bleeding disorders should monitor individuals with severe factor V deficiency.

Deterrence/Prevention

See the list below:

Factor V deficiency is an inherited disorder; no known means of prevention exists.
Immunize patients who may require plasma-derived coagulation factor concentrates with hepatitis B vaccines.

Complications

Complications of factor V deficiency are directly related to the site of bleeding (eg, hemarthrosis, intracranial hemorrhage, uncontrolled postoperative bleeding).

Prognosis

The prognosis of factor V deficiency is good with diagnosis and proper treatment.

Patient Education

See the list below:

Give patients and families instruction and educational materials to enable them to understand factor V deficiency.
For excellent patient education resources, see eMedicineHealth's patient education article Hemophilia.

Questions

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Owren P. Parahaemophilia: haemorrhagic diathesis due to absence of a previously unknown clotting factor. Lancet. 1947. Vol I:446-8.
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Liu HC, Shen MC, Eng HL, Wang CH, Lin TM. Asp68His mutation in the A1 domain of human factor V causes impaired secretion and ineffective translocation. Haemophilia. 2014 Jul. 20(4):e318-26. [QxMD MEDLINE Link].
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Liu S, Luo S, Yang L, Jin Y, Xie H, Xie Y, et al. A novel homozygous mutation (Gly1715Ser) causing hereditary factor V deficiency in a Chinese patient. Blood Coagul Fibrinolysis. 2019 Nov 26. [QxMD MEDLINE Link].
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Van den Berg SA, Verwer PE, Idema RN, Van Guldener C. Transient cefuroxime/metronidazole treatment induced factor V antibodies. BMJ Case Rep. 2014 Aug 19. 2014:[QxMD MEDLINE Link].
Wang X, Qin X, Yu Y, Wang R, Liu X, Ji M, et al. Acquired factor V deficiency in a patient with a urinary tract infection presenting with haematuria followed by multiple haemorrhages with an extremely low level of factor V inhibitor: a case report and review of the literature. Blood Coagul Fibrinolysis. 2017 Jun. 28 (4):334-341. [QxMD MEDLINE Link]. [Full Text].
Hamedani AG, Cole JW, Cheng Y, Sparks MJ, O'Connell JR, Stine OC, et al. Factor V Leiden and Ischemic Stroke Risk: The Genetics of Early Onset Stroke (GEOS) Study. J Stroke Cerebrovasc Dis. 2011 Nov 17. [QxMD MEDLINE Link].
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. 2005 Jan. 11(1):26-30. [QxMD MEDLINE Link].
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Sacco S, Dragani A, Davi G, Carolei A. Four recurrent intracerebral haemorrhages. Cerebrovasc Dis. 2003. 16(4):435-6. [QxMD MEDLINE Link].
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Bulato C, Novembrino C, Anzoletti MB, Spiezia L, Gavasso S, Berbenni C, et al. "In vitro" correction of the severe factor V deficiency-related coagulopathy by a novel plasma-derived factor V concentrate. Haemophilia. 2018 Jul. 24 (4):648-656. [QxMD MEDLINE Link].
Gavva C, Yates SG, Rambally S, Sarode R. Transfusion management of factor V deficiency: three case reports and review of the literature. Transfusion. 2016 Jul. 56 (7):1745-9. [QxMD MEDLINE Link].
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Passarelli PC, De Angelis P, Pasquantonio G, Manicone PF, Verdugo F, D'Addona A. Management of Single Uncomplicated Dental Extractions and Postoperative Bleeding Evaluation in Patients With Factor V Deficiency: A Local Antihemorrhagic Approach. J Oral Maxillofac Surg. 2018 Jun 21. 3(3):196-8. [QxMD MEDLINE Link].
Yanagiya R, Kanouchi K, Toubai T, Yamada A, Aizawa K, Shiono Y, et al. Plasma Exchange as an Initial Treatment for Severe Bleeding Induced by Acquired Factor V Deficiency: A Case Report and Mini Literature Review. Acta Haematol. 2020 Aug 12. 12 (8):1-5. [QxMD MEDLINE Link].
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Media Gallery

Antithrombin sites of action.
Cell surface-directed hemostasis. Initially, a small amount of thrombin is generated on the surface of the tissue factor (TF)–bearing cell. Following amplification, the second burst generates a larger amount of thrombin, leading to fibrin (clot) formation. Adapted from Hoffman and Monroe, Thromb Haemost 2001, 85(6): 958-65.

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Author

Olga Kozyreva, MD Attending Physician, Division of Hematology-Oncology, St Elizabeth's Medical Center; Assistant Professor, 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 Assistant Clinical Professor of Medicine, Department of Hematology/Oncology, Lahey Clinic, Sophia Gordon Cancer Center

Karl J D'Silva, MD is a member of the following medical societies: Massachusetts Medical Society

Disclosure: Nothing to disclose.

Specialty Editor Board

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, MD, FRCPC, DTM&H Professor Emeritus of Internal Medicine and Hematology/Oncology, Emeritus Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center

Ronald A Sacher, MD, 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.

Chief Editor

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.

Additional Contributors

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.