Background: We tried to understand prevalence and clinical severity of sickle cell anemia (SCA) alone or sickle cell diseases (SCD)
with associated alpha- or beta-thalassemias in adults.
Methods: All adults with the SCA or SCD were studied.
Results: The study included 441 patients (215 females). The prevalence of SCA was significantly lower than the SCD in adults
(29.0% versus 70.9%, p<0.001). The mean age and female ratio were similar in the SCA and SCD groups (31.2 versus 30.5 years
and 52.3% versus 47.2%, p>0.05 for both, respectively). The mean body mass index was similar in both groups, too (21.5 versus
21.7 kg/m2, p>0.05, respectively). On the other hand, the total bilirubin value of the plasma was higher in the SCA, significantly (5.7
versus 4.4 mg/dL, p= 0.000). Whereas the total number of transfused units of red blood cells in their lives was similar in the SCA
and SCD groups (43.6 versus 37.1 units, p>0.05, respectively).
Conclusion: The SCA alone and SCD are severe inflammatory processes on vascular endothelium particularly at the capillary
level and terminate with an accelerated atherosclerosis and end-organ failures in early years of life. The relatively suppressed
hemoglobin S synthesis in the SCD secondary to the associated thalassemia’s may decrease sickle cell-induced chronic endothelial
damage, inflammation, edema, fibrosis, and end-organ failures. The lower prevalence of the SCA in adults and the higher total
bilirubin value of the plasma in them may indicate the relative severity of hemolytic process, vascular endothelial inflammation,
and hepatic involvement in the SCA.