RESUMO
Haptoglobin is a protein involved in protecting the body from the harmful effects of free hemoglobin. The haptoglobin gene exhibits a polymorphism, and the different genotypes do not have the same capacity to combat the free hemoglobin effects. The present study aimed at determining the polymorphic distribution of haptoglobin in sickle cell patients (SCPs) from West Cameroon and their impact on the hematological parameters, as well as clinical manifestations of the disease severity. Haptoglobin genotype of 102 SCPs (SS) and 115 healthy individuals (60 AA and 55 AS) was determined by allele-specific polymerase chain reaction, and the complete blood count was determined using the AutoAnalyser. Results showed that the genotype Hp2-2 was significantly (p < 0.05) represented in SS patients (54%) than in controls AA and AS (27% and 29%, respectively), while Hp2-1 was mostly found (p < 0.05) in AS (42%) and AA (38%), against 15% in SS. The allelic distribution in SS patients was Hp2: 0.613, Hp1S: 0.304, and Hp1F: 0.084. In AA and AS controls, the proportions of the Hp1 and Hp2 alleles were similar (around 0.5 each), with 0.282 for Hp1S and 0.218 for Hp1F in AS and 0.283 for Hp1S and 0.258 for Hp1F in AA. The distribution of the haptoglobin genotypes did not reveal any significant difference across hematological parameters and clinical manifestations of disease severity in SCP and controls. SCP with Hp1S-1F genotype presented the highest level of hemoglobin. Although Hp2-2 was more frequent in SS patients, it appeared not to be related to the hematological parameters and to the disease's severity. Further investigations are necessary to explore the impact of Hp polymorphism such as antioxidant, lipid profile, and functionality of some tissues in SCP in Cameroon.
RESUMO
BACKGROUND: Several plant extracts from Rutaceae family are currently used to the management of sickle cell disorder (SCD) in the African. Few reports have shown that extracts from Zanthoxyllum or Fagara genus demonstrated anti-sickling property. This study investigates the in vitro antisickling and antioxidant properties of extracts from Zanthoxyllum heitzii. METHODS: The sickling of red blood cells (RBCs) was induced using sodium metabisulfite (2%) followed by treatment with extracts at different concentrations. The osmotic fragility tests permits to explore the effect of Z. heitzii extracts on haemoglobin S solubility and sickle cells membrane stability. For each extract, qualitative phytochemical tests were used to identify the presence of alkaloids, tannins, saponins, flavonoids, glycosides and phenols, while some quantitative methods such as Folin, Ferric Reducing Antioxidant Power (FRAP) and diphenyl 1, 2 picryl hydrazyl (DPPH) were used to determine the antioxidant potential of these extracts. RESULTS: Sodium metabisulphite increased the sickling of RBCs from 29.62 to 55.46% during 2 h. Treatment of sickling cells with extracts at different concentrations showed that a decrease of the percentage of sickling cells was found in both induced and non induced sickling cells. The fruits extract of Z. heitzii demonstrated the best anti-sickling property. The same extract at 250 µg/mL showed the best membrane cell stability compared to others. All the extracts revealed an antioxidant and anti-radical activities although lesser compared to the standard. CONCLUSION: The fruit extract of Z. Heitzii demonstrated the most significant antisickling effect with a potential for use in the clinical management of SCD.
