D55.1

HCC Category Mapping

What This Code Means

A rare type of anemia resulting from problems with glutathione metabolism, which is important for protecting red blood cells from oxidative damage. This leads to reduced red blood cell survival and anemia.

Coding Tips

• •This is a rare condition; ensure documentation clearly supports the specific glutathione metabolism disorder
• •Differentiate from G6PD deficiency (D55.0) and other enzyme disorders

Clinical Significance

This rare hemolytic anemia results from defects in glutathione metabolism pathways other than Glucose-6-phosphate dehydrogenase deficiency. Glutathione is critical for protecting red blood cells from oxidative damage, and deficiency leads to chronic or episodic hemolysis. These conditions are typically inherited in an autosomal recessive pattern and may present with neonatal jaundice, chronic hemolytic anemia, or intermittent crisis episodes.

Documentation Requirements

• ✓Documentation must specify the glutathione metabolism disorder identified (e.g., glutathione synthetase deficiency, gamma-glutamylcysteine synthetase deficiency), supported by enzyme assays or genetic testing.
• ✓Record hemoglobin levels, reticulocyte counts, evidence of hemolysis (elevated lactate dehydrogenase, indirect bilirubin, haptoglobin), and the management plan including folic acid supplementation and transfusion history.

Commonly Confused Codes