Would Prothrombin Time (PT)/International Normalized Ratio (INR) be normal in patients with Factor V Leiden?

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PT/INR in Factor V Leiden

Yes, PT/INR is normal in patients with Factor V Leiden. Factor V Leiden does not affect the extrinsic or common coagulation pathways measured by PT/INR testing 1, 2.

Why PT/INR Remains Normal

  • PT/INR measures specific coagulation factors (II, V, VII, X, and fibrinogen) in the extrinsic and common pathways, but Factor V Leiden is a qualitative defect, not a quantitative deficiency 2.

  • The mutation affects protein C resistance, not factor V levels or PT/INR. Factor V Leiden involves a single point mutation (G1691A) that substitutes glutamine for arginine at position 506, making activated Factor V resistant to inactivation by activated protein C 1.

  • Factor V is present at normal levels in patients with Factor V Leiden—the protein is simply resistant to degradation by activated protein C, which is not detected by standard PT/INR testing 3, 4.

Appropriate Testing for Factor V Leiden

  • Direct DNA-based genotyping or Factor V Leiden-specific functional assays are the recommended diagnostic methods, not PT/INR 1.

  • Modified activated protein C resistance (APC-R) assays using patient plasma diluted in Factor V-deficient plasma provide reliable differentiation between heterozygotes and homozygotes with minimal interference from other clinical factors 1, 3.

  • Traditional aPTT-based functional tests cast a wider net but lack specificity and are subject to interference from acute phase reactants, pregnancy, oral contraceptives, lupus anticoagulant, heparin, and warfarin therapy 1.

  • Patients on anticoagulation therapy should proceed directly to molecular testing if modified functional assays are not available, as standard coagulation tests cannot reliably detect Factor V Leiden in these circumstances 1, 3.

Critical Clinical Pitfall

  • Never use PT/INR as a screening test for Factor V Leiden or other inherited thrombophilias. PT/INR is only validated for monitoring vitamin K antagonist therapy and does not detect qualitative coagulation defects like Factor V Leiden 5, 2.

  • The INR therapeutic range (2.0-3.0) for anticoagulation applies equally to patients with Factor V Leiden who develop venous thromboembolism, as moderate-intensity anticoagulation is adequate for these patients 1, 6.

References

Guideline

Guideline Directed Topic Overview

Dr.Oracle Medical Advisory Board & Editors, 2025

Guideline

Deflazacort's Effect on Coagulation Pathways

Praxis Medical Insights: Practical Summaries of Clinical Guidelines, 2025

Research

Laboratory assessment of Activated Protein C Resistance/Factor V-Leiden and performance characteristics of a new quantitative assay.

Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for Haemapheresis, 2017

Guideline

Investigation of Elevated INR

Praxis Medical Insights: Practical Summaries of Clinical Guidelines, 2025

Research

Role of thrombophilia in deciding on the duration of anticoagulation.

Seminars in thrombosis and hemostasis, 2004

Professional Medical Disclaimer

This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

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