1. Increased leucocyte apoptosis in transfused β-thalassaemia patients
- Author
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Janet L. Kwiatkowski, Robert W. Grady, Daniele Alberti, Elliott Vichinsky, Annie Higa, Patricia J. Giardina, Patricia Evans, John B. Porter, Paul Harmatz, Felicia Trachtenberg, Thomas D. Coates, Bruce N. Ames, Patrick B. Walter, Ellen B. Fung, Ellis J. Neufeld, and Nancy F. Olivieri
- Subjects
Adult ,Male ,medicine.medical_specialty ,Blood transfusion ,Adolescent ,medicine.medical_treatment ,Apoptosis ,DNA Fragmentation ,Gastroenterology ,Article ,Young Adult ,Bcl-2-associated X protein ,Internal medicine ,Leukocytes ,medicine ,Humans ,Blood Transfusion ,Chelation therapy ,Child ,bcl-2-Associated X Protein ,Hematology ,biology ,business.industry ,beta-Thalassemia ,Deferasirox ,Beta thalassemia ,medicine.disease ,Chelation Therapy ,Deferoxamine ,Caspases ,Child, Preschool ,Immunology ,biology.protein ,Female ,business ,medicine.drug - 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.
- Published
- 2012
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