Estudo da correlação entre os níveis de hemoglobina fetal e o prognóstico dos pacientes com anemia falciforme

Silva, Lilianne B.; Gonçalves, Raquel; Martins, Michele F.

doi:10.1590/s1516-84842009005000084

Cited by 8 publications

(11 citation statements)

References 8 publications

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“…Studies have shown that this drug leads to increased Hb F levels in approximately 50% of patients with SCD. High Hb F concentrations may decrease the severity of illness because of inhibition in the polymerization of Hb S. Hb F alter contact sites between Hb molecules, impairing polymer formation with consequent reduction in the sickling process (21-23) . This is a potential reduction factor of hemolysis and the consequent cell damage caused by substances released during this process, for example, iron.…”

Section: Discussionmentioning

confidence: 99%

The influence of hydroxyurea on oxidative stress in sickle cell anemia

Torres

Silva

Junior

et al. 2012

Revista Brasileira De Hematologia E Hemoterapia

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Objective The oxidative stress in 20 sickle cell anemia patients taking hydroxyurea and 13 sickle cell anemia patients who did not take hydroxyurea was compared with a control group of 96 individuals without any hemoglobinopathy. MethodsOxidative stress was assessed by thiobarbituric acid reactive species production, the Trolox-equivalent antioxidant capacity and plasma glutathione levels. ResultsThiobarbituric acid reactive species values were higher in patients without specific medication, followed by patients taking hydroxyurea and the Control Group (p < 0.0001). The antioxidant capacity was higher in patients taking hydroxyurea and lower in the Control Group (p = 0.0002 for Trolox-equivalent antioxidant capacity and p < 0.0292 for plasma glutathione). Thiobarbituric acid reactive species levels were correlated with higher hemoglobin S levels (r = 0.55; p = 0.0040) and lower hemoglobin F concentrations(r = -0.52; p = 0.0067). On the other hand, plasma glutathione levels were negatively correlated with hemoglobin S levels (r = -0.49; p = 0.0111) and positively associated with hemoglobin F values (r = 0.56; p = 0.0031). ConclusionSickle cell anemia patients have high oxidative stress and, conversely, increased antioxidant activity. The increase in hemoglobin F levels provided by hydroxyurea and its antioxidant action may explain the reduction in lipid peroxidation and increased antioxidant defenses in these individuals.

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Section: Discussionmentioning

confidence: 99%

The influence of hydroxyurea on oxidative stress in sickle cell anemia

Torres

Silva

Junior

et al. 2012

Revista Brasileira De Hematologia E Hemoterapia

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“…Several studies show an association of the clinical heterogeneity ofSCD with Hb F levels and the intensity of the hemolytic process (3,18,19) . …”

Section: Discussionmentioning

confidence: 99%

Correlation of low levels of nitrite and high levels of fetal hemoglobin in patients with sickle cell disease at baseline

Elias¹,

Rocha²,

Cavalcante³

et al. 2012

Revista Brasileira De Hematologia E Hemoterapia

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Background Sickle cell disease is a hemoglobinopathy characterized by hemolytic anemia, increased susceptibility to infections and recurrent vaso-occlusive crises that reduces the quality of life of sufferers. Objective To evaluate the correlation of the levels of lactate dehydrogenase, malonaldehyde and nitrite to fetal hemoglobin in patients with sickle cell disease not under treatment with hydroxyurea in outpatients at a university hospital in Fortaleza, Ceará, Brazil. Methods Forty-four patients diagnosed with sickle cell disease were enrolled at baseline. Diagnosis was confirmed by evaluating the beta globin gene using polymerase chain reaction-restriction fragment length polymorphism. The concentration of fetal hemoglobin was obtained by high-performance liquid chromatography. Serum levels of nitrite, malonaldehyde and lactate dehydrogenase were measured by biochemical methods. Results Significantly higher levels of lactate dehydrogenase, nitrite and malonaldehyde were observed in patients with sickle cell disease compared to a control group. The study of the correlation between fetal hemoglobin levels and these variables showed a negative correlation with nitrite levels. No correlation was found between fetal hemoglobin and malonaldehyde or lactate dehydrogenase. When the study population was stratified according to fetal hemoglobin levels, a decrease in the levels of nitrite was observed with higher levels of fetal hemoglobin (p-value = 0.0415). Conclusion The results show that, similar to fetal hemoglobin levels, the concentration of nitrite can predict the clinical course of the disease, but should not be used alone as a modulator of prognosis in patients with sickle cell disease.

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“…There is a need to understand the mechanisms responsible for hemoglobin switching, as reversing it can provide major therapeutic benefits to persons affected by ␤ hemoglobinopathies [8]. Individuals with SCA and the CAR haplotype often have HbF levels below 5%, while those with the Benin haplotype show intermediary HbF levels, 5-10% [9,65]. The present study shows that the Bantu haplotype had a greater DI than Benin haplotype.…”

Section: Discussionmentioning

confidence: 56%

“…Genetic characteristics affecting clinical severity include variations in the HbF level, the coexistence of alpha-thalassemia, and the haplotype associated with the HbS gene [5][6][7][8][9][10]. The different haplotypes of SCA are Bantu or Central African Republic (CAR), Benin (BEN), Senegal (SEN), Cameroon, and Arab-Indian.…”

Section: Introductionmentioning

confidence: 99%

DNA damage in leukocytes of sickle cell anemia patients is associated with hydroxyurea therapy and with HBB*S haplotype

Rocha

Elias

Barbosa

et al. 2012

Mutation Research/Genetic Toxicology and Environmental Mutagene

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Hydroxyurea (HU) is the primary pharmacologic agent for preventing the complications and improving the quality of life of sickle cell anemia (SCA) patients. Although HU has been associated with an increased risk of leukemia in some patients with myeloproliferative disorders, the mutagenic and carcinogenic potential of HU has not been established. This study used the alkaline comet assay to investigate DNA damage in peripheral blood leukocytes from 41 individuals with SCA treated with HU (SCAHU) and from 26 normal individuals. The presence of HbS and the analysis of the haplotypes of the beta S gene cluster were done by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP). The damage index (DI) in the SCAHU group was significantly higher than in controls (p<0.001). Neither gender nor age was associated with DNA damage in controls or SCAHU individuals. Among the SCAHU individuals, DI was significantly influenced by length of HU treatment (p=0.0039) and BMI (p=0.001). Individuals with length of HU treatment≥20 months and BMI≤20kg/m(2) had a significantly greater DI than those with length of HU treatment<20 months and BMI>20kg/m(2). No significant influence of mean HU dose was observed on DI (p=0.950). However, individuals who received a mean HU dose≥20mg/kg showed a higher DI than those who received less. Furthermore, an association was observed between DI damage and HBB*S gene haplotypes. DI values for the Bantu/Bantu haplotype was greater when compared to the Benin/Benin haplotype; and the Bantu/Benin haplotype had a DI lower than the Bantu/Bantu haplotype and greater than the Benin/Benin haplotype. Our results show that DNA damage in sickle cell anemia is associated not only with treatment with HU but also with genotype.

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Estudo da correlação entre os níveis de hemoglobina fetal e o prognóstico dos pacientes com anemia falciforme

Cited by 8 publications

References 8 publications

The influence of hydroxyurea on oxidative stress in sickle cell anemia

The influence of hydroxyurea on oxidative stress in sickle cell anemia

Correlation of low levels of nitrite and high levels of fetal hemoglobin in patients with sickle cell disease at baseline

DNA damage in leukocytes of sickle cell anemia patients is associated with hydroxyurea therapy and with HBB*S haplotype

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