Other primary thrombophilia

This code signifies a congenital or inherited predisposition to abnormal blood clot formation (thrombosis) that is not due to a specific, well-defined thrombophilia like Factor V Leiden or Protein C deficiency. It encompasses various less common or unclassified genetic defects affecting coagulation pathways, leading to an increased risk of venous or arterial thromboembolism.

Apply this code when documentation indicates a primary, inherited thrombophilia that doesn't fit a more specific ICD-10-CM code. This is typically used after genetic testing or extensive workup has ruled out common thrombophilias but a genetic predisposition is still suspected or confirmed. Supporting documentation should clearly state "other primary thrombophilia" or describe a genetic thrombotic disorder not otherwise specified.

• Do not use this code if a more specific primary thrombophilia (e.g., Factor V Leiden, Protein S deficiency) is identified; use the appropriate specific code.
• Ensure documentation specifies "primary" or "inherited" to differentiate from acquired thrombophilias (e.g., due to malignancy or antiphospholipid syndrome).
• Consider sequencing this code as a secondary diagnosis if the patient is being treated for an acute thrombotic event, with the acute event coded as the primary diagnosis.