Premawardhena, A.de Silva, S.Arambepola, M.Olivieri, N. F.Vichinsky, E. P.Merson, L.Muraco, G.Allen, A.Fisher, C.Peto, T.Weatherall, D. J.2014-10-292014-10-292005Annals of the New York Academy of Sciences. 2005; 1054: pp.33-90077-8923 (Print)1749-6632 (Electronic)http://repository.kln.ac.lk/handle/123456789/1701Indexed in MEDLINEA long-term observational study of Hb E-beta-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.beta-Thalassemiabeta-Thalassemia-epidemiologybeta-Thalassemia-classificationbeta-Thalassemia-geneticsbeta-Thalassemia-bloodbeta-Thalassemia-therapySri Lanka-epidemiologyHemoglobin EHemoglobin E-geneticsReviewMedicine