Acute chest syndrome (ACS) is the result of various inciting events causing vaso-occlusion within the pulmonary vasculature of patients with sickle cell disease (SCD). ACS can occur in any SCD phenotype. However, it is most common in HbSS. ACS can progress quickly and is the most common cause of death in patients with SCD. Therefore, it is important to make the diagnosis and begin treatment as soon as possible.
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