Isolated Severe Prolongation of Prothrombin Time Revealing Congenital Factor VII Deficiency With Mild Bleeding Phenotype: A Case Report

Mohammed Alkhanafsa¹, Osayd Mosleh¹, Jamil Wafi¹, Mohammed Alkahtib¹

Keywords: Factor VII deficiency, isolated prolonged prothrombin time, INR, coagulation disorder, mild bleeding phenotype, case report

DOI: 10.63475/yjm.v5i2.0395

DOI URL: https://doi.org/10.63475/yjm.v5i2.0395

Publish Date: 25-06-2026

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Abstract

Congenital Factor VII (FVII) deficiency is a rare inherited bleeding disorder characterized by marked variability in bleeding severity and poor correlation between factor activity levels and clinical phenotype. We report a 21-year-old woman who presented with recurrent spontaneous ecchymoses without mucosal bleeding, menorrhagia, hemarthrosis, or prior excessive surgical bleeding. Laboratory evaluation demonstrated markedly prolonged prothrombin time (PT) of 96 seconds with an international normalized ratio (INR) of 8.0 and normal activated partial thromboplastin time (aPTT). Repeat testing confirmed persistent isolated PT prolongation. There was no evidence of liver disease, vitamin K deficiency, anticoagulant exposure, disseminated intravascular coagulation, systemic illness, or malignancy. A PT mixing study completely corrected the abnormal PT, supporting a factor deficiency rather than an inhibitor. Specific factor assays demonstrated isolated severe FVII deficiency with activity <5.6%, while Factors II, IX, and X were normal. Despite severe laboratory abnormalities, the patient had only mild cutaneous bleeding manifestations and had previously undergone uneventful septorhinoplasty. She was managed conservatively with education and individualized follow-up. This case highlights the important discordance between PT/INR severity and bleeding phenotype in congenital FVII deficiency. This case highlights extreme PT/INR prolongation with severe FVII deficiency but mild phenotype and prior uneventful surgery, emphasizing that INR should not be interpreted like warfarin-associated coagulopathy in congenital FVII deficiency.

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