Other hemolytic anemias due to enzyme deficiency

This code signifies a group of inherited hemolytic anemias resulting from a deficiency in specific enzymes crucial for red blood cell metabolism, excluding G6PD deficiency (which has its own specific code). These enzyme defects lead to premature destruction of red blood cells, causing anemia.

Assign this code when documentation specifies a hemolytic anemia caused by an enzyme deficiency other than glucose-6-phosphate dehydrogenase (G6PD) deficiency. Examples include pyruvate kinase deficiency, hexokinase deficiency, or triosephosphate isomerase deficiency. Supporting documentation should include laboratory findings confirming the specific enzyme defect and evidence of hemolytic anemia.

• Do not use this code for G6PD deficiency; refer to category 282.2.
• Ensure the physician documentation clearly identifies the specific enzyme deficiency if known, or at least rules out G6PD.
• Code any associated symptoms or complications of the anemia, such as splenomegaly or jaundice, as secondary diagnoses.