Increased leucocyte apoptosis in transfused β-thalassaemia patients

Br J Haematol. 2013 Feb;160(3):399-403. doi: 10.1111/bjh.12076. Epub 2012 Dec 6.

Abstract

This exploratory study assessed apoptosis in peripheral blood leucocytes (PBL) from β-thalassaemia patients receiving chronic transfusions and chelation therapy (deferasirox or deferoxamine) at baseline, 1, 6, and 12 months. At baseline, thalassaemic PBLs presented 50% greater levels of Bax (BAX), 75% higher caspase-3/7, 48% higher caspase-8 and 88% higher caspase-9 activities and 428% more nucleosomal DNA fragmentation than control subjects. Only neutrophils correlated significantly with apoptotic markers. Previously, we showed that over the treatment year, hepatic iron declined; we now show that the ratio of Bax/Bcl-2 (BCL2), (-27·3%/year), and caspase-9 activity (-13·3%/year) declined in both treatment groups, suggesting that chelation decreases body iron and indicators of PBL apoptosis.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Adolescent
  • Adult
  • Apoptosis*
  • Blood Transfusion
  • Caspases / metabolism
  • Chelation Therapy
  • Child
  • Child, Preschool
  • DNA Fragmentation
  • Female
  • Humans
  • Leukocytes / metabolism*
  • Male
  • Young Adult
  • bcl-2-Associated X Protein / metabolism
  • beta-Thalassemia / metabolism*
  • beta-Thalassemia / therapy

Substances

  • bcl-2-Associated X Protein
  • Caspases