Plasma Levels of Advanced Glycation End Products are Associated with Hemolysis-related Organ Complications in Sickle Cell Patients

Erfan Nur (1) , D. P.M. Brandjes (2) , John J. Schnog (3) , Hans-Martin Otten (4) , Karin Fijnvandraat (5) , Casper G. Schalkwijk (6) , Bart J Biemond (7)

1 Hematology
2 Dept. of Internal Medicine
3 Dept. of Haematology/Oncology
4 Internal Medicine
5 Department of Paediatric Clinical Epidemiology
6 Laboratory for Metabolism and Vascular Medicine
7 Dept. of Haematology

Oxidative stress plays an important role in the pathophysiology of sickle cell disease (SCD). Plasma levels of advanced glycation end products (AGEs) are increased under oxidative conditions and are associated with disease severity in diabetes and inflammatory diseases. We questioned whether AGEs are increased in sickle cell patients and whether they are associated with SCD related complications. Plasma levels of the AGEs pentosidine, Nε-(carboxymethyl)lysine (CML) and Nε-(carboxyethyl)lysine (CEL)) were measured using single-column high performance liquid chromatography with fluorescence detection (pentosidine) and ultra performance liquid chromatography-tandem mass spectrometry (CML and CEL). Plasma levels of pentosidine and CML were increased in HbSS/HbSβ0-thalassemia (n=60) and HbSC/HbSβ+-thalassemia (n=42) patients during steady state as compared to healthy HbAA controls (n=30) without increments during painful crisis. CEL levels were comparable between all groups. Pentosidine and CML levels correlated significantly to hemolytic rate during the clinically asymptomatic state while pentosidine was significantly related to the number of hemolysis-related organ complications. The increased plasma AGE levels in sickle cell patients and their association with hemolysis and hemolysis-related complications suggest AGEs might be implicated in the pathophysiology of the hemolytic phenotype of SCD. Measurement of AGEs might be useful in predicting organ complications in SCD.

Mots clés: Medicine

Erfan Nur, D. P.M. Brandjes, John J. Schnog, Hans-Martin Otten, Karin Fijnvandraat, et al.. Plasma Levels of Advanced Glycation End Products are Associated with Hemolysis-related Organ Complications in Sickle Cell Patients. British Journal of Haematology, 2010, 151 (1), pp.62. ⟨10.1111/j.1365-2141.2010.08320.x⟩. ⟨hal-00560772⟩