title: Prevalence of glutathione S-transferase gene deletions and their effect on sickle cell patients.
abstract: BACKGROUND
  Glutathione S-transferase gene deletions are known detoxification agents and cause oxidative damage. Due to the different pathophysiology of anemia in thalassemia and sickle cell disease, there are significant differences in the pathophysiology of iron overload and iron-related complications in these disorders.
  
  
  OBJECTIVE
  The aim of this study was to estimate the frequency of the GSTM1 and GSTT1 genotypes in sickle cell disease patients and their effect on iron status.
  
  
  METHODS
  Forty sickle cell anemia and sixty sickle ß-thalassemia patients and 100 controls were evaluated to determine the frequency of GST gene deletions. Complete blood counts were performed by an automated cell analyzer. Hemoglobin F, hemoglobin A, hemoglobin A2 and hemoglobin S were measured and diagnosis of patients was achieved by high performance liquid chromatography with DNA extraction by the phenol-chloroform method. The GST null genotype was determined using multiplex polymerase chain reaction and serum ferritin was measured using an ELISA kit. Statistical analysis was by EpiInfo and GraphPad statistics software.
  
  
  RESULTS
  An increased frequency of the GSTT1 null genotype (p-value = 0.05) was seen in the patients. The mean serum ferritin level was higher in patients with the GST genotypes than in controls; this was statistically significant for all genotypes except GSTM1, however the higher levels of serum ferritin were due to blood transfusions in patients.
  
  
  CONCLUSIONS
  GST deletions do not play a direct role in iron overload of sickle cell patients.
authors.affiliation: Department of Hematology, All India Institute of Medical Sciences - AIIMS, New Delhi, India.
authors.forename: Pandey
authors.initials: P
authors.lastname: Sanjay
authors.forename: Mishra Rahasy
authors.initials: MR
authors.lastname: Mani
authors.forename: Pandey
authors.initials: P
authors.lastname: Sweta
authors.forename: Shah
authors.initials: S
authors.lastname: Vineet
authors.forename: Ahuja Rajesh
authors.initials: AR
authors.lastname: Kumar
authors.forename: Saxena
authors.initials: S
authors.lastname: Renu