D57.411

Billable

Sickle-cell thalassemia, unspecified, with acute chest syndrome

HCC Category Mapping

V28HCC 108 — Sickle Cell Disorders and Thalassemia

0.607

V24HCC 46 — Severe Hematological Disorders

0.666

ESRDHCC 46 — Severe Hematological Disorders

0.000

What This Code Means

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

Coding Tips

•Acute chest syndrome in sickle-cell thalassemia is a medical emergency; ensure acute status is clearly documented
•This code indicates both the underlying disease and the acute pulmonary complication

Clinical Significance

Acute chest syndrome in unspecified sickle-cell thalassemia represents a life-threatening pulmonary complication in patients with combined sickle cell and thalassemia genes where the specific thalassemia type (beta-zero vs. beta-plus) is not documented. The clinical presentation and management are similar to acute chest syndrome in other sickle cell genotypes, with new pulmonary infiltrate, respiratory distress, and potential for rapid deterioration requiring intensive care.

Documentation Requirements

✓Documentation must confirm the sickle-cell thalassemia genotype and the clinical diagnosis of acute chest syndrome with respiratory symptoms and new pulmonary infiltrate on imaging.
✓Query the provider for beta-zero vs.
✓beta-plus thalassemia specification.
✓Record oxygen saturation, treatment including transfusion protocol, antibiotics, respiratory support, and clinical course.
✓The provider must explicitly diagnose acute chest syndrome.

Commonly Confused Codes

D57.411 vs. D57.431 (Sickle-cell thalassemia beta zero with acute chest syndrome) -use D57.431 when beta-zero is specifically documented. D57.411 vs. D57.451 (Sickle-cell thalassemia beta plus with acute chest syndrome) -use D57.451 when beta-plus is documented. D57.411 vs. D57.01 (Hemoglobin SS with acute chest syndrome) -different genotype.

Code Hierarchy

└D57Sickle-cell disorders └D57.4Sickle-cell thalassemia └D57.41Sickle-cell thalassemia, unspecified, with crisis └D57.411Sickle-cell thalassemia, unspecified, with acute chest syndrome

└D57.411Sickle-cell thalassemia, unspecified, with acute chest syndrome