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D57.451

Billable

Sickle-cell thalassemia beta plus with acute chest syndrome

HCC Category Mapping

V28HCC 108Sickle Cell Disorders and Thalassemia
0.607
V24HCC 46Severe Hematological Disorders
0.666
ESRDHCC 46Severe Hematological Disorders
0.000

What This Code Means

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

Coding Tips

  • Acute chest syndrome is a medical emergency; document respiratory symptoms, fever, chest pain, and imaging findings (chest X-ray or CT)
  • Distinguish from D57.431 by confirming beta-plus (not beta-zero) thalassemia is documented

Clinical Significance

Acute chest syndrome in sickle-cell thalassemia beta plus is the same life-threatening pulmonary complication seen in other sickle cell genotypes, though it may occur less frequently in beta-plus patients due to the milder overall disease phenotype. Despite the generally milder course, acute chest syndrome can still be severe and potentially fatal, requiring the same aggressive management with exchange transfusion, antibiotics, and respiratory support.

Documentation Requirements

  • Documentation must confirm the sickle-cell thalassemia beta plus genotype and the explicit diagnosis of acute chest syndrome with new pulmonary infiltrate and respiratory symptoms (chest pain, dyspnea, cough, fever, hypoxia).
  • Record oxygen saturation, imaging results, treatment protocol, and clinical course.
  • The provider must document 'acute chest syndrome' as the diagnosis.

Commonly Confused Codes

Code Hierarchy

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