title: DNA damage: beta zero versus beta plus thalassemia.
abstract: BACKGROUND
  β thalassemia results in an increase in the α to non-α chain ratio. Iron released from unpaired α chains in RBCs and that ensuing from regular transfusions is the major cause of cellular damage. The use of iron chelators to counter the iron overload is accompanied by side-effects. The extent of iron toxicity could vary from one patient to another and could help in determining the optimal chelator dose for each patient.
  
  
  OBJECTIVE
  To observe the pro-oxidant/antioxidant disturbance and the extent of DNA damage in β thalassemia patients with different β globin gene anomalies.
  
  
  METHODS
  The formation of Reactive Oxygen Species (ROS ) was observed by incubation of cell suspensions with 2',7', dichlorofluorescin-diacetate (DCFH DA) and DNA damage was demonstrated by single cell gel electrophoresis. Heinz bodies were observed by staining blood smears.
  
  
  METHODS
  The study group comprised 50 regularly transfused beta thalassemia patients and 40 non thalassemic controls.
  
  
  RESULTS
  While Heinz bodies and nucleated RBCs were seen in all the patients, oxidation of DCFH and DNA damage were seen to be associated with the β globin gene defect. DNA damage was found to be greater in β(0) homozygotes as compared to the β(+) homozygotes, and was maximum in patients presenting with the 619 base pair deletion.
  
  
  CONCLUSIONS
  In the present study, iron toxicity, as indicated by DNA damage, has been seen to vary in the patients. Thus, monitoring of the dose of iron chelators, according to the type of mutation in the beta globin gene, may help improve the compliance of beta thalassemics to chelation therapy and prevent side-effects in patients with beta plus mutations.
authors.affiliation: a School of Studies in Biochemistry , Jiwaji University , Gwalior , India and.
authors.forename: Chandan S
authors.initials: CS
authors.lastname: Sagar
authors.affiliation: a School of Studies in Biochemistry , Jiwaji University , Gwalior , India and.
authors.forename: Rakesh
authors.initials: R
authors.lastname: Kumar
authors.affiliation: b Blood Bank, Jaya Arogya Hospitals , Gajra Raja Medical College , Gwalior , India.
authors.forename: Dharmesh C
authors.initials: DC
authors.lastname: Sharma
authors.affiliation: a School of Studies in Biochemistry , Jiwaji University , Gwalior , India and.
authors.forename: Purnima
authors.initials: P
authors.lastname: Kishor
mesh_terms: D000293:Adolescent; D000975:Antioxidants / Q000378:metabolism*; D002648:Child; D002675:Child, Preschool; D020552:Comet Assay; D004249:DNA Damage; D005452:Fluoresceins / Q000378:metabolism; D006366:Heinz Bodies / Q000737:chemistry; D006801:Humans; D007194:India; D007223:Infant; D018384:Oxidative Stress; D017382:Reactive Oxygen Species / Q000097:blood*; D055815:Young Adult; D055544:beta-Globins / Q000235:genetics* / Q000378:metabolism; D017086:beta-Thalassemia / Q000503:physiopathology*
chemical_list: D000975:Antioxidants; D005452:Fluoresceins; D017382:Reactive Oxygen Species; D055544:beta-Globins; C029569:diacetyldichlorofluorescein
keywords: Alpha chain toxicity; DCFH; oxidative stress