ICD-10 Code D57.01 – Hb-SS disease with acute chest syndrome (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code D57.01 – Hb-SS disease with acute chest syndrome
What it is
D57.01 identifies sickle cell anemia, Hb-SS type, complicated by acute chest syndrome. Use it when the record shows both the underlying Hb-SS disease and this acute pulmonary complication.
Clinical signs
Acute chest syndrome usually presents with new chest symptoms, respiratory distress, or infiltrates on imaging in a patient with sickle cell disease. Clinical features vary; refer to documentation.
When to use this code
Use this code when the provider documents Hb-SS disease and acute chest syndrome during the same encounter. It may apply during inpatient care, emergency treatment, or follow-up for a recently resolved episode if still documented as active. Check documentation for whether the syndrome is current.
Do not use for
Do not use D57.01 for sickle cell disease without acute chest syndrome, or for chest pain alone without the syndrome documented. Do not assume the diagnosis from imaging or symptoms alone; check documentation.
Coding tip
Code the acute chest syndrome only when the provider clearly links it to Hb-SS disease in the record.