- Walter, Patrick B;
- Porter, John;
- Evans, Patricia;
- Kwiatkowski, Janet L;
- Neufeld, Ellis J;
- Coates, Thomas;
- Giardina, Patricia J;
- Grady, Robert W;
- Vichinsky, Elliott;
- Olivieri, Nancy;
- Trachtenberg, Felicia;
- Alberti, Daniele;
- Fung, Ellen;
- Ames, Bruce;
- Higa, Annie;
- Harmatz, Paul;
- Network, Thalassemia Clinical Research
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.