Hereditary factor XI deficiency

This code represents a rare, inherited bleeding disorder characterized by a deficiency in coagulation factor XI (plasma thromboplastin antecedent). Individuals with this condition may experience prolonged bleeding after trauma or surgery, though spontaneous bleeding is uncommon. The severity of bleeding symptoms does not always correlate with the factor XI activity level.

Use this code for patients diagnosed with congenital factor XI deficiency, often identified through abnormal activated partial thromboplastin time (aPTT) and confirmed by specific factor XI assay. Documentation should clearly state "hereditary" or "congenital" factor XI deficiency.

• Distinguish from acquired factor XI deficiency, which would be coded differently.
• Ensure documentation specifies the hereditary nature; otherwise, further clarification may be needed.
• Consider sequencing with codes for any associated bleeding manifestations if applicable.