Biomarkers for the diagnosis and phenotyping of acute chest syndrome in patients with sickle cell disease

INTRODUCTION: Acute Chest Syndrome (ACS) is a serious and often fatal complication of Sickle Cell Disease (SCD). In the absence of specific biomarkers that indicate the development of ACS, diagnosis can be nebulous and difficult. In this narrative literature review, we examine the current evidence for novel biomarkers associated with acute and long-term propensity toward ACS. AREAS COVERED: A search focused on terms related to 'acute chest syndrome,' 'sickle cell anemia,' and 'biomarkers' was created and run by a medical librarian in MEDLINE (Ovid), Google Scholar, and Embase (Ovid). Four-hundred seven articles were found in the initial search. After duplicates removed and irrelevant studies excluded, 13 were included for final data extraction. Data extraction was completed by three authors and collated to form the final manuscript. EXPERT OPINION: Currently, ACS diagnosis remains a great challenge. The reviewed literature shows promise in both novel biomarkers like sputum IL-6 and alternative interpretations of routine studies including blood counts and inflammatory markers. Still, large, multicenter trials to validate reliable and specific markers for ACS remain elusive. Isolating a dependable, rapid, and widely available and affordable biomarker for ACS may help diagnose and change morbidity and mortality from one of SCD's most fatal complications.