Background and aims: Major sickle cell syndromes are subjected to a high frequency of hemolysis, infections, oxidative stress, and vasooclusive crises which promote inflammation and iron balance disorders. We aimed to systematically review and analyze the studies in this patients addressing in general, and Africa in particular.
Methods: The systematic review of published articles in the Pubmed and Google Scholar databases was carried out according to the recommendations of the PRISMA model. The case-control articles have been included. The data extracted from the articles were analyzed using statistical software R. The standardized mean difference (SMD) was used to assess the extent of the disease on the different variables studied.
Results: At the end, 128 articles were obtained; but only 33 were elligible for meta-analysis. A SMD of -1.79 was obtained for hemoglobin between the sickle cell patients and the controls due to the deviation from the overall mean hemoglobin in the cases (8 ± 2 g/dL) and in controls (13 ± 3 g/dL). Sickle cell disease showed a significant extent on ferritin [SMD = 2.61; (95% confidence interval, CI: 2.39-2.83); (p < 0.01)] compared to non-sickle cell patients thus describing a higher risk for sickle cell sufferer to have ferritin disorders. The included studies also described the influence of sickle cell anemia on serum iron [SMD = 1.52; (95% CI: 1.32-1.76); (p < 0.01)] compared to normal subjects. The high risk of inflammation has been described as higher in sickle cell patients [SMD = 0.38; (95% CI: 0.25-0.50)], reflecting the moderate extent of sickle cell disease on inflammation.
Conclusion: Patients with major sickle cell syndrome in inflammation have a higher risk of iron profile disorders compared to the normal population. Further studies are needed to explore mechanisms for preventing the deleterious effects of iron from this hemolysis, for example haptoglobin genotyping.
Keywords: ferritin; hemoglobin; hemolysis; inflammation; serum iron; sickle cell anemia.
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