D55.3

HCC Category Mapping

What This Code Means

A type of anemia caused by problems with nucleotide metabolism, which affects the ability of red blood cells to function properly. This leads to reduced red blood cell survival and anemia.

Coding Tips

• •Nucleotide metabolism disorders are rare; ensure thorough documentation supports this diagnosis
• •Distinguish from other enzyme disorders affecting different metabolic pathways

Clinical Significance

Anemia due to nucleotide metabolism disorders represents a rare group of hereditary hemolytic anemias where defects in purine or pyrimidine metabolism impair red blood cell function and survival. The most recognized condition in this category is pyrimidine 5'-nucleotidase deficiency, which causes basophilic stippling of red blood cells and chronic hemolysis. These disorders are clinically significant due to their chronic nature and potential for complications including gallstones and iron overload.

Documentation Requirements

• ✓Documentation must identify the specific nucleotide metabolism disorder when known, with supporting laboratory evidence such as enzyme activity assays, peripheral blood smear findings (particularly basophilic stippling), and markers of hemolysis.
• ✓Record hemoglobin levels, reticulocyte counts, and any complications.
• ✓Genetic testing results should be documented if available.

Commonly Confused Codes