D57.20

HCC Category Mapping

What This Code Means

A combination of sickle cell disease and hemoglobin C disease without an acute crisis, causing chronic anemia and abnormal red blood cells in a stable state.

Coding Tips

• •Confirm both sickle hemoglobin and hemoglobin C are present through hemoglobin electrophoresis documentation
• •Use only when patient is not experiencing acute crisis; if crisis develops, use appropriate D57.2x crisis code

Clinical Significance

Sickle-cell/Hemoglobin C disease (Hemoglobin SC) is a compound heterozygous condition where the patient inherits one sickle hemoglobin gene and one hemoglobin C gene. It is the second most common sickle cell genotype and is generally milder than Hemoglobin SS disease, with higher baseline hemoglobin and less frequent pain crises. However, patients are at increased risk for retinopathy, avascular necrosis, and splenic complications including sequestration and infarction in adulthood.

Documentation Requirements

• ✓Documentation must confirm the Hemoglobin SC genotype through hemoglobin electrophoresis or genetic testing, and confirm the patient is not in acute crisis.
• ✓Record baseline hemoglobin, reticulocyte count, and current medications.
• ✓Document screening for retinopathy (annual ophthalmologic exam), avascular necrosis, and splenic function.
• ✓Note any disease-modifying therapy and vaccination status.

Commonly Confused Codes