D57.01

HCC Category Mapping

What This Code Means

This is a serious complication of sickle cell disease where a person with two copies of the sickle cell gene (HbSS) develops sudden chest pain, difficulty breathing, and lung inflammation. It's a medical emergency that requires immediate hospitalization and treatment.

Coding Tips

• •Acute chest syndrome must be documented by the physician as a current condition during the encounter; do not assume it from lab values or imaging alone
• •This code includes the sickle cell disease diagnosis, so do not separately code D57.00 (HbSS disease without crisis) when acute chest syndrome is present

Clinical Significance

Acute chest syndrome in Hemoglobin SS disease is a life-threatening complication characterized by new pulmonary infiltrate on chest imaging with respiratory symptoms including chest pain, fever, cough, or hypoxia. It is the leading cause of death and the second most common cause of hospitalization in sickle cell disease. The pathophysiology involves vaso-occlusion in pulmonary vasculature, fat embolism from bone marrow infarction, and infection, often requiring intensive care management.

Documentation Requirements

• ✓Documentation must include the Hemoglobin SS genotype, clinical presentation with respiratory symptoms (chest pain, dyspnea, cough, fever, hypoxia), chest imaging confirming new pulmonary infiltrate, and the provider's explicit diagnosis of acute chest syndrome.
• ✓Record oxygen saturation, arterial blood gas results, treatment administered (exchange transfusion, antibiotics, supplemental oxygen, bronchodilators), and clinical course.
• ✓Do not rely solely on lab values or imaging to assign this code.

Commonly Confused Codes