Hereditary hemochromatosis

This code signifies a genetic disorder characterized by excessive iron absorption and accumulation in various organs, leading to organ damage. It primarily affects the liver, heart, and pancreas, causing conditions like cirrhosis, cardiomyopathy, and diabetes.

Assign this code when documentation confirms a diagnosis of hereditary hemochromatosis, often identified through genetic testing for HFE gene mutations or elevated iron studies (ferritin, transferrin saturation). It is appropriate for initial diagnoses, ongoing management, and when complications directly stem from the iron overload.

• Do not confuse with secondary hemochromatosis (iron overload due to other conditions like transfusions); those have different codes.
• Always code any associated manifestations or complications (e.g., cirrhosis, diabetes mellitus) in addition to this code.
• Ensure documentation specifies "hereditary" or "genetic" to differentiate from acquired forms of iron overload.