D57.811

Billable

Other sickle-cell disorders 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 rare sickle-cell disorder with acute chest syndrome, a serious lung complication causing chest pain and breathing difficulty.

Coding Tips

•Acute chest syndrome is a medical emergency; ensure it is clearly documented in the clinical record
•This code indicates a crisis state, so document supporting symptoms like fever, chest pain, or respiratory distress

Clinical Significance

Other sickle-cell disorders with acute chest syndrome represents a life-threatening pulmonary complication occurring in patients with rare sickle-cell variants. Acute chest syndrome is characterized by a new pulmonary infiltrate on chest imaging accompanied by fever, chest pain, cough, or hypoxia, and is a leading cause of death in sickle-cell disease. This condition requires immediate hospitalization with aggressive hydration, pain management, transfusion support, and respiratory care.

Documentation Requirements

✓Document the specific sickle-cell variant along with chest imaging findings showing new pulmonary infiltrate.
✓Record presenting symptoms including fever, chest pain, dyspnea, cough, and oxygen saturation levels.
✓Include laboratory values such as hemoglobin, white blood cell count, and arterial blood gases.
✓Document any interventions including supplemental oxygen, exchange transfusion, antibiotics, and bronchodilators.

Commonly Confused Codes

D57.01 (Hemoglobin SS disease with acute chest syndrome) is used for homozygous sickle-cell disease specifically.D57.211 (Sickle-cell/hemoglobin C disease with acute chest syndrome) applies when hemoglobin C is the second variant.D57.411/D57.451 covers sickle-cell thalassemia variants with acute chest syndrome.Simple pneumonia (J18.x) should not be used alone when acute chest syndrome is diagnosed in a sickle-cell patient.

Code Hierarchy

└D57Sickle-cell disorders └D57.8Other sickle-cell disorders └D57.81Other sickle-cell disorders with crisis └D57.811Other sickle-cell disorders with acute chest syndrome

└D57.811Other sickle-cell disorders with acute chest syndrome