Congenital factor XI deficiency

This code identifies 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, surgery, or dental procedures, though spontaneous bleeding is less common. The severity of bleeding symptoms can vary widely among affected individuals.

Assign this code when documentation confirms a congenital deficiency of factor XI, often identified through coagulation studies showing reduced factor XI activity. This diagnosis is typically made after a patient presents with abnormal bleeding or a family history of the disorder. It is appropriate for both symptomatic and asymptomatic individuals with a confirmed genetic or laboratory diagnosis.

• Differentiate from acquired factor XI inhibitors, which would be coded differently.
• Look for specific mention of "congenital" or "hereditary" to support this code.
• Consider additional codes for any associated bleeding manifestations or complications.