Effects of glucose-6-phosphate dehydrogenase deficiency upon sickle cell anemia

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.

Impact of glucose‐6‐phosphate dehydrogenase deficiency on sickle cell anaemia expression in infancy and early childhood: a prospective study

“…Similarly, the transfusion rate was lower in the CSSCD cohort, with 35% of children being transfused before 5 years vs. 46% before 42 months for our study. The rates of infection and vaso-occlusive crisis were not significantly different between the two groups, in agreement with earlier data from cross-sectional studies (Bienzle et al, 1975;Gibbs et al, 1980;Steinberg et al, 1988;Bouanga et al, 1998;Nouraie et al, 2010). Despite that, G6PD-deficient patients had an almost 3-fold increase in blood transfusion requirement for both AAEs and vaso-occlusive or infectious events.…”

“…Studies investigating potential effects of G6PD deficiency on the severity of SCA have produced conflicting results. Some studies found no effect of G6PD deficiency on the clinical manifestations, Hb levels, or reticulocyte counts of SCA patients (Gibbs et al, 1980;Steinberg et al, 1988), whereas others found lower Hb levels in patients having both diseases (Bouanga et al, 1998;Nouraie et al, 2010). In a study of transcranial Doppler ultrasonography (TCD) findings in a cohort of stroke-free SCA patients, G6PD deficiency was among the independent risk factors for high blood flow velocities, a finding predictive of stroke (Bernaudin et al, 2008).…”

“…Controversial results were reported with lack of statistical significance on baseline haematological parameters recorded mainly at a single point in time, either in SCA populations covering a very broad age range (Gibbs et al, 1980;Bouanga et al, 1998;Rees et al, 2009;Nouraie et al, 2010) or after 12-36 months of age (Steinberg et al, 1988;Bernaudin et al, 2008;Sheehan et al, 2013).…”

Advances in understanding the pathogenesis of cerebrovascular vasculopathy in sickle cell anaemia

“…The strength of the association between G6PD and SCArelated morbidity is controversial. Previous studies (Steinberg et al, 1988) found no evidence that G6PD deficiency could increase the severity of haemolysis or the incidence of acute anaemic episodes in SCA.…”

Sickle cell disease in Saudi Arabia: the phenotype in adults with the Arab‐Indian haplotype is not benign

“…Summary of multivariate regressions of HbF on historical outcomes, adjusted for gender and age at enrollment. a-Thalassaemia 51% n/a n/a 62% 42% (Adekile et al, 2007) 38% 30% (Steinberg & Embury, 1986) G6PD deficiency 28% n/a n/a 21% 2Á5% 10% (Steinberg et al, 1988) SCD, sickle cell disease; G6PD, glucose-6-phosphate dehydrogenase; MCV, mean corpuscular volume; n/a, not reported or not applicable.…”