D58.1
BillableHereditary elliptocytosis
HCC Category Mapping
What This Code Means
A genetic blood disorder where red blood cells are abnormally shaped like ellipses or ovals instead of the normal disc shape, causing them to break down faster than normal.
Coding Tips
- •Confirm the hereditary nature of the condition in documentation
- •Note any associated hemolytic episodes or complications
Clinical Significance
Hereditary elliptocytosis is a group of inherited red blood cell membrane disorders where the cells assume an elliptical or oval shape due to defects in spectrin, protein 4.1, or glycophorin C. Most patients are asymptomatic carriers with mild or no hemolysis, but a subset develops clinically significant hemolytic anemia, particularly those with homozygous or compound heterozygous mutations. Severe forms (hereditary pyropoikilocytosis) can present in infancy with transfusion-dependent anemia.
Documentation Requirements
- ✓Document the hereditary nature with family history when available.
- ✓Record peripheral blood smear findings showing elliptocytes (typically greater than 25% of red blood cells).
- ✓Include hemoglobin level, reticulocyte count, and markers of hemolysis.
- ✓Document disease severity and whether the patient is symptomatic or an asymptomatic carrier.
- ✓Note any history of splenectomy or transfusion requirements.