Correlation Between the Frequency of Painful Crises in Sickle Cell Anemia and the Biomarkers of Inflammation, Endothelial Dysfunction, Coagulation Activation and Bone Metabolism

Objective: Sickle cell anemia is a widespread inherited hemolytic anemia, characterized by chronic hemolysis, infections, recurrent occlusion of the microcirculation and painful crises. In this cross-sectional study, we investigated the correlation between biomarkers of inflammation, endothelial dysfunction, coagulation activation and bone metabolism, with the frequency of painful crisis in sickle cell anemia adult patients, to understand the potential role of these biomarkers in the management of sickle cell anemia. Methods: Sixty-three sickle cell anemia patients were enrolled in this study. They were divided into three groups based on the frequency of pain crises to mildly, moderately, and severely affected. Twenty-five healthy volunteers were enrolled as controls. Plasma levels of different biomarkers were tested, including tumor necrosis factor alpha, soluble intercellular adhesion molecule-1, D-dimer, sclerostin, and nitric oxide. Results: Tumor necrosis factor alpha, soluble intercellular adhesion molecule-1, and D-Dimer plasma levels were significantly higher in sickle cell anemia patients when compared to the controls, while nitric oxide levels were notably lower in the patients' group. Sickle cell anemia patients with severe pain showed higher tumor necrosis factor alpha, soluble intercellular adhesion molecule-1 and D-dimer levels than those mildly and moderately affected with painful crisis. Positive correlations were observed between all measured biomarkers and the frequency of the painful crisis, except for nitric oxide, which showed a negative correlation. Conclusion: Tumor necrosis factor alpha, soluble intercellular adhesion molecule-1, nitric oxide, and D-dimer can be used to assess sickle cell anemia severity and prognosis, and thus may affect determining appropriate management strategies.