Laboratory Studies

The presence of a mild prolongation of the prothrombin time (PT) and activated partial thromboplastin time (aPTT) may be the first indication of factor V deficiency. Coagulation study results are as follows:

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

Use specific factor V activity and antigen assays for confirmation. The factor V antigen assay 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.^[24]However, this test is not routinely ordered to diagnose factor V deficiency. A factor V inhibitor panel should also be ordered.

Imaging Studies

Early and aggressive imaging studies are indicated, even with low suspicion for hemorrhage, after coagulation therapy is initiated, and may include the following:

Head computed tomography (CT) scan (noncontrast) - To assess spontaneous or traumatic hemorrhage
Body CT scan - Perform as indicated by clinical suspicion and anatomical location, to assess spontaneous or traumatic hemorrhage. May be performed with or without intravenous contrast, oral contrast, or both.
Head and spinal column MRI - To 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.

Treatment & Management

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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.

Factor V Deficiency Workup

Laboratory Studies

Imaging Studies

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