Acute Chest Syndrome in Sickle Cell Disease

Acute chest syndrome (ACS) is the leading cause of mortality among individuals with sickle cell disease (SCD) accounting for 25% of all deaths. The etiologies and clinical manifestations of ACS are variable among children and adults, with a lack of clear risk stratification guidelines for the practicing clinician. In addition, the management of ACS is based on limited evidence and is currently guided primarily by expert opinion. This manuscript reviews the pathophysiology, risk factors, and current management strategies for ACS through a review of published data on this subject between 1988 and 2022. Blood transfusion is often used as a therapeutic intervention for ACS to increase blood's oxygen-carrying capacity and reduce complications by reducing hemoglobin S (HbS) percentage, based on the very low quality of the evidence about its efficacy. The benefit of RBC transfusion for ACS has been described in case series and observational studies, but randomized studies comparing simple transfusion vs. exchange transfusions for ACS are lacking. In this review, we conclude that the development of clinical and laboratory risk stratification is necessary to further study an optimal management strategy for individuals with ACS to avoid transfusion-related complications while minimizing mortality.