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Emerging insights in the management of hemoglobin E beta thalassemia
Author(s) -
Olivieri Nancy F.,
Thayalsuthan Vivekanandan,
O’Donnell Angela,
Premawardhena Anuja,
Rigobon Christopher,
Muraca Giulia,
Fisher Christopher,
Weatherall David J.
Publication year - 2010
Publication title -
annals of the new york academy of sciences
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.712
H-Index - 248
eISSN - 1749-6632
pISSN - 0077-8923
DOI - 10.1111/j.1749-6632.2010.05579.x
Subject(s) - beta thalassemia , thalassemia , hemoglobin , medicine , beta (programming language) , blood transfusion , intensive care medicine , computer science , programming language
Globally, hemoglobin (Hb) E β thalassemia accounts for approximately half the severe forms of β thalassemia. Because of its wide clinical diversity and the ability of patients with this condition to adapt unusually well to low hemoglobin levels, the management of Hb E β thalassemia, particularly the decision to instigate regular blood transfusion, is particularly difficult. Here, we present a summary of our work in patients with this condition, which attempts to define clinical, adaptive, and genetic factors of possible value in determining the early management of this condition.