Hemoglobin E-β-thalassemia: Progress report from the International Study Group
Premawardhena A., De Silver S., Arambepola M., Olivieri NF., Vichinsky EP., Merson L., Muraco G., Allen A., Fisher C., Peto T., Weatherall DJ.
A long-term observational study of Hb E-β-thalassemia in Sri Lanka is beginning to define some of the genetic and environmental factors that are responsible for its remarkable phenotypic variability. In this population there is a very small difference between the steady-state hemoglobin levels between the mild and severe phenotypes, and it has been possible to stop transfusion in many of those who have been on long-term treatment of this kind. These preliminary observations, made over the last 7 years, provide directions for future research into this increasingly important disease. © 2005 New York Academy of Sciences.