Related Experiment Videos
Effects of glucose-6-phosphate dehydrogenase deficiency upon sickle cell anemia
M H Steinberg1, M S West, D Gallagher
1Jackson VA Medical Center MS 39216.
Insights
Glucose-6-phosphate dehydrogenase (G6PD) deficiency does not significantly alter the clinical or laboratory features of sickle cell anemia (HbSS). This study found no evidence that G6PD deficiency worsens hemolysis or increases complications in HbSS patients.
Area of Science:
- Hematology
- Genetics
- Biochemistry
Background:
- Sickle cell anemia (HbSS) is a severe inherited blood disorder.
- Glucose-6-phosphate dehydrogenase (G6PD) deficiency is a common inherited enzyme disorder.
- The interaction between G6PD deficiency and HbSS requires further investigation.
Purpose of the Study:
- To investigate the influence of G6PD deficiency on the laboratory and clinical manifestations of HbSS.
- To determine if G6PD deficiency exacerbates hemolysis or increases complications in HbSS patients.
Main Methods:
- Studied 801 male patients over age 2 with HbSS.
- Performed G6PD electrophoresis, G6PD activity assays, and hexokinase activity assays.
- Collected clinical data using a standardized protocol and employed cluster analyses.
Main Results:
- 10.4% of males were classified as G6PD deficient; 18.4% had the GdA+ enzyme.
- No significant differences were observed in hemoglobin levels, MCV, reticulocyte counts, bilirubin, or SGOT between G6PD deficient and non-deficient HbSS patients.
- The incidence of painful episodes, sepsis, and acute anemic episodes was similar in both groups.
Conclusions:
- G6PD deficiency does not appear to significantly influence the laboratory or clinical features of HbSS.
- There is no evidence that G6PD deficiency enhances hemolysis or increases the risk of complications in HbSS.
- Findings align with smaller studies suggesting minimal impact of G6PD deficiency on HbSS severity.
Abstract:
We studied the interactions of the A- variety of glucose-6-phosphate dehydrogenase (G6PD) deficiency and sickle cell anemia (HbSS) to see if G6PD deficiency influenced laboratory and clinical features of HbSS. A total of 801 male patients over age 2 had G6PD electrophoresis on cellulose acetate membranes. Assays of both G6PD activity and hexokinase activity were then done on all samples that had an electrophoretic pattern other than the normal wild type (GdB). The collection of clinical data used a standardized protocol. Using cluster analyses we classified 10.4% males to be G6PD deficient, while 18.4% had the functionally normal GdA+ enzyme. The prevalence of G6PD deficiency did not change significantly when age was stratified by decade, suggesting little survival advantage or disadvantage of the combination of G6PD deficiency and HbSS. Compared to patients who were not G6PD deficient, there were no significant differences in the hemoglobin concentration, mean corpuscular volume, reticulocyte count, bilirubin, or SGOT level in patients with HbSS who had G6PD deficiency. The incidence of painful episodes, sepsis, or acute anemic episodes was similar in both groups. Our results are consistent with recent studies of smaller numbers of patients that have found little influence of G6PD deficiency upon HbSS. Specifically, we found no evidence that G6PD enhanced the severity of hemolysis or increased the incidence of acute anemic episodes or sepsis in HbSS.