Abstract:ObjectiveThis study aimed to evaluate the influence of fetal hemoglobin (Hb F) on hemolysis biomarkers in sickle cell anemia patients.MethodsFifty adult sickle cell anemia patients were included in the study. All patients were taking hydroxyurea for at least six months and were followed at the outpatient clinic of a hospital in Fortaleza, Ceará, Brazil. The control group consisted of 20 hemoglobin AA individuals. The reticulocyte count was performed by an automated methodology, lactate dehydrogenase and uric a… Show more
“…HbF levels were positively correlated with hemoglobin, hematocrit, MCV and total protein levels, in addition to negatively correlated with MCHC, RDW, as well as AST and AAT levels. A previous study demonstrated a negative correlation between HbF levels and hemolytic biomarkers, reticulocytes and LDH levels, in individuals with SCA [34]. Although we did not find this correlation, our results also suggest that the increases in HbF levels induced by HU are followed by improvements in the hemolytic and inflammatory profiles of the children with SCA studied herein.…”
This study investigated the effects of hydroxyurea (HU) on hematological, biochemical and inflammatory parameters in children with sickle cell anemia (SCA) in association with β
S
haplotype and α-thalassemia. We included 22 children with SCA who were followed for an average of 14.5 months. Laboratory parameters were assessed by electronic methods, and molecular analysis was investigated by PCR-RFLP and allele-specific PCR. Results showed significant increases in hemoglobin, HbF, hematocrit, MCV, MCH, glucose, HDL-C and albumin levels, as well as significant decreases in MCHC and AST levels, WBC, neutrophils, eosinophils, lymphocytes and reticulocytes, in children during HU therapy. HbF levels were positively correlated with hemoglobin, hematocrit, MCV and total protein, yet negatively correlated with MCHC, RDW, AAT and AST during HU therapy (
p
<0.05). Children who carried the Central African Republic haplotype, in response to HU therapy, presented significant increases in hemoglobin, hematocrit, triglycerides and uric acid levels, as well as significant decreases in MCHC, AST and direct bilirubin levels, WBC, neutrophils, eosinophils, lymphocytes and reticulocytes. Those with the Benin haplotype presented increases in HbF and albumin levels, and a reduction in platelet counts (
p
<0.05). Children with α-thalassemia presented decreased ALT during HU use, while those without this deletion presented increases in hemoglobin, hematocrit, MCV, MCH, HDL-C and albumin, as well as decreases in MCHC, neutrophils, lymphocytes, reticulocytes and AST (
p
<0.05). Hence, regardless of its use in association with β
S
haplotypes or α-thalassemia, HU seems to be linked to alterations in hemolytic, inflammatory, hepatic, lipid and glycemic profiles.
“…HbF levels were positively correlated with hemoglobin, hematocrit, MCV and total protein levels, in addition to negatively correlated with MCHC, RDW, as well as AST and AAT levels. A previous study demonstrated a negative correlation between HbF levels and hemolytic biomarkers, reticulocytes and LDH levels, in individuals with SCA [34]. Although we did not find this correlation, our results also suggest that the increases in HbF levels induced by HU are followed by improvements in the hemolytic and inflammatory profiles of the children with SCA studied herein.…”
This study investigated the effects of hydroxyurea (HU) on hematological, biochemical and inflammatory parameters in children with sickle cell anemia (SCA) in association with β
S
haplotype and α-thalassemia. We included 22 children with SCA who were followed for an average of 14.5 months. Laboratory parameters were assessed by electronic methods, and molecular analysis was investigated by PCR-RFLP and allele-specific PCR. Results showed significant increases in hemoglobin, HbF, hematocrit, MCV, MCH, glucose, HDL-C and albumin levels, as well as significant decreases in MCHC and AST levels, WBC, neutrophils, eosinophils, lymphocytes and reticulocytes, in children during HU therapy. HbF levels were positively correlated with hemoglobin, hematocrit, MCV and total protein, yet negatively correlated with MCHC, RDW, AAT and AST during HU therapy (
p
<0.05). Children who carried the Central African Republic haplotype, in response to HU therapy, presented significant increases in hemoglobin, hematocrit, triglycerides and uric acid levels, as well as significant decreases in MCHC, AST and direct bilirubin levels, WBC, neutrophils, eosinophils, lymphocytes and reticulocytes. Those with the Benin haplotype presented increases in HbF and albumin levels, and a reduction in platelet counts (
p
<0.05). Children with α-thalassemia presented decreased ALT during HU use, while those without this deletion presented increases in hemoglobin, hematocrit, MCV, MCH, HDL-C and albumin, as well as decreases in MCHC, neutrophils, lymphocytes, reticulocytes and AST (
p
<0.05). Hence, regardless of its use in association with β
S
haplotypes or α-thalassemia, HU seems to be linked to alterations in hemolytic, inflammatory, hepatic, lipid and glycemic profiles.
“…This result is in agreement with previous studies, which reported the anti-sickling effect of HbF on individual RBC 18 , 54 , which increases RBC deformability 55 . HU treatment has been also shown to decrease haemolysis and free hemoglobin in plasma 7 , 56 – 60 . Figure 4 The adjusted viscosity at Hct = 45% (filled circles) and HVR (crosses) for SCD blood.…”
Sickle cell disease (SCD) is an inherited blood disorder associated with severe anemia, vessel occlusion, poor oxygen transport and organ failure. The presence of stiff and often sickle-shaped red blood cells is the hallmark of SCD and is believed to contribute to impaired blood rheology and organ damage. Most existing measurement techniques of blood and red blood cell physical properties require sample contact and/or large sample volume, which is problematic for pediatric patients. Acoustic levitation allows rheological measurements in a single drop of blood, simultaneously eliminating the need for both contact containment and manipulation of samples. The technique shows that the shape oscillation of blood drops is able to assess blood viscosity in normal and SCD blood and demonstrates an abnormally increased viscosity in SCD when compared with normal controls. Furthermore, the technique is sensitive enough to detect viscosity changes induced by hydroxyurea treatment, and their dependence on the total fetal hemoglobin content of the sample. Thus this technique may hold promise as a monitoring tool for assessing changes in blood rheology in sickle cell and other hematological diseases.
“…Moreira et al 7 also demonstrated that hydroxyurea therapy and subsequent elevation of Hb F levels was associated to reductions in arginase I levels. The lowest arginase I levels were seen in patients taking hydroxyurea at doses greater than 20 mg/kg/day.…”
mentioning
confidence: 94%
“…In the current issue of the Revista Brasileira de Hematologia e Hemoterapia, there is an article entitled “Pattern of hemolysis parameters and association with fetal hemoglobin in sickle cell anemia patients in steady state” by Moreira et al 7 In this article the authors demonstrate the beneficial effects of elevated Hb F levels on hemolysis markers in SCA patients. When patients achieved Hb F levels above 10% they presented with significant reductions in reticulocyte counts and lactate dehydrogenase (LDH) levels.…”
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