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guides & guidelines

Management of acute complications of sickle cell disease

key information

source: American Society of Hematology

year: 2014

authors: Timothy McCavit, Payal Desai


Acute chest syndrome (ACS): ACS resembles pneumonia, e.g., the patient has signs and symptoms of lower respiratory tract disease and a new pulmonary infiltrate on chest radiograph. ACS can develop suddenly or insidiously. Patients may present with ACS, or it may occur during hospitalization for a vaso-occlusive crisis or after a surgical procedure, especially one involving the abdomen. Risk is increased in people with asthma or prior ACS events. ACS is the most common cause of death in adults with SCD. Evaluation should include chest x-ray and measurement of oxygen saturation by pulse oximetry. It should also include CBC/ reticulocyte count and blood culture.

Management: Treat with intravenous cephalosporin and an oral macrolide antibiotic. If the patient is hypoxemic, administer supplemental oxygen to maintain >95% oxygen saturation. Monitor closely for bronchospasm, acute anemia, and hypoxemia. Consider simple blood transfusion (10 mL/kg RBCs) if Hb concentration is >1.0 g/dL below baseline (Weak recommendation, low-quality evidence). If baseline Hb is ≥9 g/dL, simple transfusion may not be indicated (Weak recommendation, low-quality evidence). Avoid transfusing to a target Hb >10 g/dL (Moderate recommendation, low-quality evidence). Perform urgent exchange transfusion if there is rapid progression of ACS as manifested by <90% oxygen saturation, increasing respiratory distress, progressive pulmonary infiltrates, and/or decline in haemoglobin concentration. Encourage use of incentive spirometry while awake.

organization: University of Texas Southwestern Medical Center; The Ohio State University

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