ICD-10 Code D57.431 – Sickle-cell thalassemia beta zero with acute chest syndrome (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code D57.431 – Sickle-cell thalassemia beta zero with acute chest syndrome
What it is
This code identifies sickle-cell thalassemia beta zero with an episode of acute chest syndrome. It combines a hereditary hemoglobin disorder with a serious pulmonary complication requiring specific documentation.
Clinical signs
Clinical features vary; refer to documentation. Acute chest syndrome is typically documented with chest pain, fever, cough, shortness of breath, or new pulmonary findings in a patient with sickle-cell disease.
When to use this code
Use this code when the record clearly states sickle-cell thalassemia beta zero and also documents acute chest syndrome. The diagnosis should be supported by the provider’s assessment, not inferred from nonspecific respiratory symptoms alone.
If the note says “acute chest syndrome” without specifying beta zero thalassemia, check documentation for the exact sickle-cell subtype before assigning this code.
Do not use for
Do not use it for sickle-cell thalassemia without acute chest syndrome, or for other sickle-cell variants documented instead. Check documentation if the subtype or complication is unclear.
Coding tip
Verify both elements in the same encounter note: the beta zero thalassemia subtype and the acute chest syndrome diagnosis.