D57.431

HCC Category Mapping

What This Code Means

A combination blood disorder with sickle-cell and thalassemia beta-zero that causes acute chest syndrome, a serious lung complication with chest pain and breathing difficulty.

Coding Tips

• •Acute chest syndrome is a medical emergency; ensure documentation clearly describes respiratory symptoms, chest pain, and imaging findings
• •Verify beta-zero thalassemia is specifically documented; beta-plus thalassemia uses code D57.451 for the same complication

Clinical Significance

Acute chest syndrome in sickle-cell thalassemia beta zero carries the same life-threatening risk as in Hemoglobin SS disease because the beta-zero genotype produces a clinically equivalent phenotype. These patients develop new pulmonary infiltrates with respiratory symptoms and may deteriorate rapidly, requiring exchange transfusion and intensive care. The recognition of beta-zero status is important because management intensity mirrors Hemoglobin SS protocols.

Documentation Requirements

• ✓Documentation must confirm sickle-cell thalassemia beta zero genotype and the explicit diagnosis of acute chest syndrome with new pulmonary infiltrate and respiratory symptoms. Record oxygen saturation, arterial blood gas values, chest imaging findings, treatment (exchange transfusion targeting Hemoglobin S <30%, antibiotics, respiratory support), and clinical course. The provider must document 'acute chest syndrome' -
• ✓do not infer from imaging.

Commonly Confused Codes