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Acute and chronic inflammatory processes and concomitant disturbances in cell adhesion characterize the pathogenesis of sickle cell disease. To investigate these processes further, we analyzed serum levels of highly sensitive C-reactive protein (CRP), vascular cell adhesion molecule-1 (VCAM-1), intracellular cell adhesion molecule-1 (ICAM-1), and interleukin (IL)-1, IL-2, IL-6, IL-8, and IL-12 in a homogenous cohort of individuals with double heterozygous sickle cell/-thalassemia. We found that steady-state serum levels of CRP and VCAM-1 demonstrated statistically significant correlations with the clinical severity index in this cohort (P = .01), while other markers showed no associations. Although the mechanisms underlying observed associations between serum CRP and VCAM-1 levels and adverse outcomes have yet to be elucidated, their measurement during the course of sickle cell/-thalassemia may guide and predict disease evolution and outcome. Furthermore, therapies that reduce these levels in the steady state of sickle cell disease may be clinically useful.

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AUTHOR INFORMATION
Alexandros C. Makis, MD, PhD; Eleftheria C. Hatzimichael, MD; Justin Stebbing, MD, MRCP, PhD; Konstantinos L. Bourantas, MD, PhD