Inflammatory Biomarkers and Cardiovascular Complications in Sickle Cell Disease: A Review

Sickle cell disease (SCD) is an inheritable genetic disorder hallmarked by a single point mutation in the β-globin chain resulting in abnormal polymerization of deoxygenated hemoglobin and subsequent sickling of the erythrocyte. Longevity in SCD patients has greatly increased, shifting the paradigm for cause of death from acute to chronic conditions. Endothelial dysfunction attributed to SCD triggers a cascade of inflammatory biomarkers known to mediate several cardiovascular complications, however, the etiology of heart disease in SCD remains unclear. Cardiovascular manifestations resulting in premature mortality include elevated cardiac output, systolic and diastolic dysfunction, pulmonary hypertension, and sudden cardiac death. This review explores relevant biomarkers of inflammation typically observed as mediators or indicators of cardiovascular disease (CVD) as well as current literature on cardiovascular complications found in those with SCD. Additionally, the potential role of CVD treatments to manage the severity of SCD will be explored. © 2013 Springer Science+Business Media New York.