Hemoglobin E-β-Thalassemia: Progress Report from the International Study Group Conference Paper uri icon

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abstract

  • A 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.

authors

  • Muraca, Giulia
  • PREMAWARDHENA, ANUJA
  • SILVER, SHANTHIMALA
  • ARAMBEPOLA, MAHINDA
  • OLIVIERI, NANCY F
  • VICHINSKY, ELLIOTT P
  • MERSON, LAURA
  • MURACO, GIULIA
  • ALLEN, ANGELA
  • FISHER, CHRISTOPHER
  • PETO, TIMOTHY
  • WEATHERALL, DAVID J

publication date

  • November 2005

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