Abstract
The present study aimed to investigate the association of N (ε) -carboxymethyllysine (CML) with laboratory parameters and β (S) haplotypes in pediatric sickle cell anemia (SCA) patients with or without hydroxyurea (HU) therapy. We included 55 children with SCA (SCA(total)), where 27 were on HU treatment (SCA-HU(+)) and 28 without HU treatment (SCA-HU(-)). Laboratory characteristics were determined using electronic methods while CML was measured using competitive ELISA. β (S) haplotypes were determined by RFLP-PCR. Significant increases in MCV and MCH and significant decreases in leukocytes, eosinophils, basophils, atypical lymphocytes, lymphocytes, and monocytes were found in SCA-HU(+) compared to SCA-HU(-). SCA-HU(+) presented significant reduction in aspartate transaminase and lactate dehydrogenase and increase in creatinine levels compared to SCA-HU(-). CML levels were significantly higher in both SCA-HU(+) and SCA-HU(-) compared to the healthy control. In addition, a negative correlation was found between CML and alanine transaminase in SCA-HU(+) and SCA(total) (p < 0.01). A significant association was found between CML levels and β (S) haplotypes. The results suggest that CML has a role to play in SCA complications, independent of HU therapy.