Anemia due to pyruvate kinase deficiency

This code identifies a specific type of hemolytic anemia caused by an inherited deficiency of the enzyme pyruvate kinase. This deficiency impairs the red blood cells' ability to produce energy, leading to their premature destruction and subsequent anemia. Patients typically present with chronic anemia, jaundice, and splenomegaly.

Use this code when documentation clearly indicates a diagnosis of anemia specifically attributed to pyruvate kinase deficiency. This diagnosis is often confirmed through enzyme assays demonstrating reduced pyruvate kinase activity in red blood cells, along with genetic testing. It is appropriate for both congenital and acquired forms of the deficiency.

• Ensure documentation explicitly states "pyruvate kinase deficiency" as the cause of anemia, not just "hemolytic anemia."
• Do not confuse this with other enzyme deficiencies causing hemolytic anemia, such as G6PD deficiency (D55.0).
• Code any associated manifestations, such as splenomegaly (R16.1) or jaundice (R17), as secondary diagnoses.