Identifying the silent deficiency: Severe Factor XI deficiency in an asymptomatic patient with isolated prolonged...

Assessing a patient with a suspected bleeding disorder is a complex aspect of hematology. Prolonged activated partial thromboplastin time often indicates a coagulation factor deficiency or inhibitor and warrants detailed evaluation. Factor XI deficiency, a rare autosomal recessive disorder, shows variable bleeding tendencies with poor correlation between factor levels and bleeding risk. We report a 24-year-old asymptomatic male with an incidentally prolonged activated partial thromboplastin time during preoperative assessment for septoplasty. He had no bleeding or family history suggestive of a bleeding disorder. Mixing studies showed full correction, and factor assays confirmed severe Factor XI deficiency. Given the uncertain bleeding phenotype, a tailored perioperative plan was implemented, including fresh frozen plasma and prophylactic tranexamic acid. Recombinant activated Factor VII was available as rescue therapy, but not required. The surgery and postoperative course were uneventful, highlighting the importance of individualized perioperative management in Factor XI deficiency.