D57.211

HCC Category Mapping

What This Code Means

A blood disorder combining sickle cell disease and hemoglobin C disease that causes a sudden, life-threatening complication affecting the lungs with chest pain and difficulty breathing.

Coding Tips

• •Acute chest syndrome is a serious vaso-occlusive crisis; ensure documentation clearly indicates this acute complication occurred during the encounter
• •This code requires documentation of both the sickle-cell/Hb-C disease AND the acute chest syndrome to be appropriately assigned

Clinical Significance

Acute chest syndrome in sickle-cell/Hemoglobin C disease presents similarly to Hemoglobin SS acute chest syndrome but may be less frequent. It remains a serious, potentially life-threatening complication requiring new pulmonary infiltrate with respiratory symptoms. Hemoglobin SC patients may have a deceptively higher baseline hemoglobin that can mask the severity of acute illness, and hyperviscosity can exacerbate pulmonary complications.

Documentation Requirements

• ✓Documentation must confirm the Hemoglobin SC genotype, the clinical diagnosis of acute chest syndrome with respiratory symptoms (chest pain, dyspnea, fever, hypoxia), and new pulmonary infiltrate on chest imaging. Record oxygen saturation, treatment administered (transfusion or exchange transfusion, antibiotics, oxygen), and clinical course. The provider must explicitly diagnose acute chest syndrome -
• ✓do not infer from imaging alone.

Commonly Confused Codes