Miguel Sierra Hoffman, MD

Abstract: Acute chest syndrome (ACS) is one of the most common causes of death and hospitalization among patients with a sickle hemoglobinopathy. The clinical presentation is characterized by the appearance of a new infiltrate on a chest radiograph, with 1 or more new symptoms, including fever, cough, chest pain, and dyspnea. Additional findings include leukocytosis, hypoxemia, and auscultatory signs of consolidation. The differential diagnosis includes pneumonia, pulmonary infarction, fat embolism syndrome, pulmonary edema, and bone infarction. Treatment of ACS involves supportive care, empiric antibiotic therapy, and red blood cell transfusion when indicated. The decision of whether to use simple or exchange transfusions depends on the severity of illness and the risk of acute respiratory failure. Currently, hydroxyurea is the only FDA-approved drug designated as a preventive therapy. (J Respir Dis. 2005;26(12):529-534)