Background: Blood transfusion (BT) is essential in treating sickle cell disease (SCD); however, it leads to iron overload (IO) and oxidative stress. We studied the relationship between oxidative stress, iron status parameters, hepcidin mRNA gene expression, and IO in SCD patients. Methods: We classified all SCD patients (n = 90) into two groups: Group I, 45 children (s.ferr itin 938 ng/mL) and Group II, 45 children (s.ferritin < 938 ng/mL). A total of 55 children, age and sex matched, participated as a control group. Malondialdehyde (MDA), nitrite, s.iron, s.total iron-binding capacity (sTIBC), transferrin saturation %, s.ferritin, s.hepcidin, and hepcidin mRNA gene expression were assessed. Results: Among SCD BT-dependent patients (>3 times/year), 63% were from Group I and 37% from Group II, p < .01. The two patient groups had significantly lower s.hepcidin and hepcidin gene expression than controls (p < .001). TIBC, s.iron, s.ferritin, transferrin saturation %, ferritin/ hepcidin ratio, and MDA levels were higher among SCD patients than controls (p < .001).
Group I had higher mean level of ferritin/hepcidin ratio and MDA than Group II (p < .01). The higher level of MDA and increased frequency of BT were the significant predicting risk factors for IO (p < .05). A receiver-operating characteristic curve indicates that MDA is the outstanding significant biomarker for high level of s.ferritin with subsequent IO progression. Conclusion: MDA may serve as a biomarker of oxidative stress and IO in SCD patients. This result paid attention for urgent initiation of antioxidant and chelation therapy on detecting increased MDA level.
Elbostany, Eman A.; Elghoroury, Eman A.; Thabet, Eman H.; Rashad, Alaa A.; Rasheed, Enas A.; El-Saeed, Gamila S.M.; Abdelhalim, Dalia A.; Abdelfattah, Safa N.; Salama, Iman I.; and Salama, Niveen
"Oxidative stress and hepcidin expression in pediatric sickle cell anemia with iron overload,"
Hematology/Oncology and Stem Cell Therapy: Vol. 16
, Article 8.
Available at: https://doi.org/10.1016/j.hemonc.2021.11.003
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