Hemoglobin E‐β‐Thalassemia: Progress Report from the International Study Group Conferences

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abstract

Abstract: 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.

authors

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

status

published

publication date

November 2005

has subject area

Adolescent (MeSH)
Adult (MeSH)
Blood Transfusion (MeSH)
Case Management (MeSH)
Child (MeSH)
Child, Preschool (MeSH)
Combined Modality Therapy (MeSH)
Erythropoietin (MeSH)
Female (MeSH)
General Science & Technology (Science Metrix)
Genetic Heterogeneity (MeSH)
Hemoglobin E (MeSH)
Hemoglobins (MeSH)
Humans (MeSH)
Infant (MeSH)
International Cooperation (MeSH)
Iron Overload (MeSH)
Longitudinal Studies (MeSH)
Male (MeSH)
Middle Aged (MeSH)
Phenotype (MeSH)
Pregnancy (MeSH)
Pregnancy Complications, Hematologic (MeSH)
Severity of Illness Index (MeSH)
Splenectomy (MeSH)
Sri Lanka (MeSH)
Transfusion Reaction (MeSH)
beta-Thalassemia (MeSH)

published in

Annals of the New York Academy of Sciences Journal

presented at event

8th Cooleys Anemia Symposium Conference