Effect of N(Epsilon)-(carboxymethyl)lysine on Laboratory Parameters and Its Association with β(S) Haplotype in Children with Sickle Cell Anemia

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.